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Identification Taxonomy Ontology Physical properties Spectra Biological properties Concentrations Links References enzymes (17)transporters (4) Show 21 proteins XML

enzymes (17)transporters (4) Show 21 proteins

Record Information

Version

5.0

Status

Detected and Quantified

Creation Date

2005-11-16 15:48:42 UTC

Update Date

2021-09-14 15:20:01 UTC

HMDB ID

HMDB0000161

Secondary Accession Numbers

HMDB00161

Metabolite Identification

Common Name

L-Alanine

Description

Alanine (Ala), also known as L-alanine is an alpha-amino acid. These are amino acids in which the amino group is attached to the carbon atom immediately adjacent to the carboxylate group (alpha carbon). Amino acids are organic compounds that contain amino (‚ÄìNH2) and carboxyl (‚ÄìCOOH) functional groups, along with a side chain (R group) specific to each amino acid. L-alanine is one of 20 proteinogenic amino acids, i.e., the amino acids used in the biosynthesis of proteins. Alanine is found in all organisms ranging from bacteria to plants to animals. It is classified as an aliphatic, non-polar amino acid. In humans, alanine is a non-essential amino acid that can be easily made in the body from either the conversion of pyruvate or the breakdown of the dipeptides carnosine and anserine. Alanine can be also synthesized from branched chain amino acids such as valine, leucine, and isoleucine. Alanine is produced by reductive amination of pyruvate through a two-step process. In the first step, alpha-ketoglutarate, ammonia and NADH are converted by the enzyme known glutamate dehydrogenase to glutamate, NAD+ and water. In the second step, the amino group of the newly-formed glutamate is transferred to pyruvate by an aminotransferase enzyme, regenerating the alpha-ketoglutarate, and converting the pyruvate to alanine. The net result is that pyruvate and ammonia are converted to alanine. In mammals, alanine plays a key role in glucose‚Äìalanine cycle between tissues and liver. In muscle and other tissues that degrade amino acids for fuel, amino groups are collected in the form of glutamate by transamination. Glutamate can then transfer its amino group to pyruvate, a product of muscle glycolysis, through the action of alanine aminotransferase, forming alanine and alpha-ketoglutarate. The alanine enters the bloodstream and is transported to the liver. The alanine aminotransferase reaction takes place in reverse in the liver, where the regenerated pyruvate is used in gluconeogenesis, forming glucose which returns to the muscles through the circulation system. Alanine is highly concentrated in muscle and is one of the most important amino acids released by muscle, functioning as a major energy source. Plasma alanine is often decreased when the BCAA (branched-chain amino acids) are deficient. This finding may relate to muscle metabolism. Alanine is highly concentrated in meat products and other high-protein foods like wheat germ and cottage cheese. Alanine is an important participant as well as a regulator of glucose metabolism. Alanine levels parallel blood sugar levels in both diabetes and hypoglycemia, and alanine is reduced in both severe hypoglycemia and the ketosis of diabetes. Alanine is an important amino acid for lymphocyte reproduction and immunity. Alanine therapy has helped dissolve kidney stones in experimental animals. Normal alanine metabolism, like that of other amino acids, is highly dependent upon enzymes that contain vitamin B6. Alanine, like GABA, taurine, and glycine, is an inhibitory neurotransmitter in the brain (http://www.dcnutrition.com/AminoAcids/).

Structure

MOL 3D MOL SDF 3D SDF PDB 3D PDB SMILES InChI

View in JSmol View Stereo Labels

Synonyms

Value	Source
(2S)-2-Aminopropanoic acid	ChEBI
(S)-2-Aminopropanoic acid	ChEBI
(S)-Alanine	ChEBI
A	ChEBI
Ala	ChEBI
ALANINE	ChEBI
L-2-Aminopropionic acid	ChEBI
L-Alanin	ChEBI
L-alpha-Alanine	ChEBI
(2S)-2-Aminopropanoate	Generator
(S)-2-Aminopropanoate	Generator
L-2-Aminopropionate	Generator
L-a-Alanine	Generator
L-Α-alanine	Generator
(S)-(+)-Alanine	HMDB
(S)-2-Amino-propanoate	HMDB
(S)-2-Amino-propanoic acid	HMDB
2-Aminopropanoate	HMDB
2-Aminopropanoic acid	HMDB
2-Aminopropionate	HMDB
2-Aminopropionic acid	HMDB
2-Ammoniopropanoate	HMDB
2-Ammoniopropanoic acid	HMDB
a-Alanine	HMDB
a-Aminopropionate	HMDB
a-Aminopropionic acid	HMDB
alpha-Alanine	HMDB
alpha-Aminopropanoate	HMDB
alpha-Aminopropanoic acid	HMDB
alpha-Aminopropionate	HMDB
alpha-Aminopropionic acid	HMDB
L-(+)-Alanine	HMDB
L-2-Aminopropanoate	HMDB
L-2-Aminopropanoic acid	HMDB
L-a-Aminopropionate	HMDB
L-a-Aminopropionic acid	HMDB
L-alpha-Aminopropionate	HMDB
L-alpha-Aminopropionic acid	HMDB
Abufène	HMDB
Alanine doms-adrian brand	HMDB
Alanine, L-isomer	HMDB
Doms-adrian brand OF alanine	HMDB
Doms adrian brand OF alanine	HMDB
L-Isomer alanine	HMDB
Alanine, L isomer	HMDB
L Alanine	HMDB

Chemical Formula

C₃H₇NO₂

Average Molecular Weight

89.0932

Monoisotopic Molecular Weight

89.047678473

IUPAC Name

(2S)-2-aminopropanoic acid

Traditional Name

L-alanine

CAS Registry Number

56-41-7

SMILES

C[C@H](N)C(O)=O

InChI Identifier

InChI=1S/C3H7NO2/c1-2(4)3(5)6/h2H,4H2,1H3,(H,5,6)/t2-/m0/s1

InChI Key

QNAYBMKLOCPYGJ-REOHCLBHSA-N

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as alanine and derivatives. Alanine and derivatives are compounds containing alanine or a derivative thereof resulting from reaction of alanine at the amino group or the carboxy group, or from the replacement of any hydrogen of glycine by a heteroatom.

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Carboxylic acids and derivatives

Sub Class

Amino acids, peptides, and analogues

Direct Parent

Alanine and derivatives

Alternative Parents

Substituents

Alanine or derivatives
Alpha-amino acid
L-alpha-amino acid
Amino acid
Carboxylic acid
Monocarboxylic acid or derivatives
Hydrocarbon derivative
Organic oxygen compound
Primary amine
Organooxygen compound
Organonitrogen compound
Organic nitrogen compound
Primary aliphatic amine
Carbonyl group
Amine
Organopnictogen compound
Organic oxide
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

proteinogenic amino acid (CHEBI:16977 )
L-alpha-amino acid (CHEBI:16977 )
alanine (CHEBI:16977 )
pyruvate family amino acid (CHEBI:16977 )
Common amino acids (C00041 )

Ontology

Physiological effect

Adverse health effect

Digestive system disorder

Gastrointestinal disorder

Upper GI Disorder

Eosinophilic esophagitis

Lower GI Disorder

Irritable bowel syndrome

Genetic disorder

Inborn errors of metabolism

Glucagon deficiency - Reduced in human blood (PMID: 904979 ).
N-acetylglutamate synthetase deficiency - Elevated in human blood (PMID: 7623444 ).
Dimethylglycine dehydrogenase deficiency

Organic acidemia

3-phosphoglycerate dehydrogenase deficiency
Pyruvate carboxylase deficiency
Sarcosinemia

Poisoning

Paraquat poisoning - Elevated in human urine (PMID: 31506554 ).

Circulatory system disorder

Cardiac disorder

Heart failure - Elevated in human blood (PMID: 16789974 ).

Blood and lymphatic system disorder

Hyperglycinemia

Psychiatric disorder

Schizophrenia - Elevated in human blood (PMID: 2415198 ).

Disposition

Biological location

Excreta

Cellular substructure

Macromolecular complex

Ingestion

Process

Naturally occurring process

Physical Properties

State

Solid

Experimental Molecular Properties

Property	Value	Reference
Melting Point	300 °C	Not Available
Boiling Point	Not Available	Not Available
Water Solubility	204 mg/mL	Human Metabolome Project
LogP	-2.85	SANGSTER (1994)

Experimental Spectral Properties

Not Available

Predicted Molecular Properties

Property	Value	Source
Water Solubility	447 g/L	ALOGPS
logP	10^(-3) g/L	ALOGPS
logP	10^(-2.8) g/L	ChemAxon
logS	10^(0.7) g/L	ALOGPS
pKa (Strongest Acidic)	2.47	ChemAxon
pKa (Strongest Basic)	9.48	ChemAxon
Physiological Charge	0	ChemAxon
Hydrogen Acceptor Count	3	ChemAxon
Hydrogen Donor Count	2	ChemAxon
Polar Surface Area	63.32 Å²	ChemAxon
Rotatable Bond Count	1	ChemAxon
Refractivity	20.5 m³·mol⁻¹	ChemAxon
Polarizability	8.49 Å³	ChemAxon
Number of Rings	0	ChemAxon
Bioavailability	Yes	ChemAxon
Rule of Five	Yes	ChemAxon
Ghose Filter	No	ChemAxon
Veber's Rule	No	ChemAxon
MDDR-like Rule	No	ChemAxon

Predicted Spectral Properties

Predicted Collision Cross Sections

Predictor	Adduct Type	CCS Value (Å²)	Reference
DarkChem	[M+H]+	116.777	31661259
DarkChem	[M-H]-	112.602	31661259
AllCCS	[M+H]+	125.589	32859911
AllCCS	[M-H]-	123.356	32859911

Predicted Kovats Retention Indices

Derivatized

Derivative Name / Structure	SMILES	Kovats RI Value	Column Type	Reference
L-Alanine,1TMS,#1	C[C@H](N)C(=O)O[Si](C)(C)C	944.7	Semi standard non polar	https://arxiv.org/abs/1905.12712
L-Alanine,1TMS,#2	C[C@H](N[Si](C)(C)C)C(=O)O	1060.9	Semi standard non polar	https://arxiv.org/abs/1905.12712
L-Alanine,1TBDMS,#1	C[C@H](N)C(=O)O[Si](C)(C)C(C)(C)C	1167.5	Semi standard non polar	https://arxiv.org/abs/1905.12712
L-Alanine,1TBDMS,#2	C[C@H](N[Si](C)(C)C(C)(C)C)C(=O)O	1337.0	Semi standard non polar	https://arxiv.org/abs/1905.12712

Spectra

GC-MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Experimental GC-MS	GC-MS Spectrum - L-Alanine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (1 TMS)	splash10-014i-0900000000-c7f6dbace291e8305a4e	2014-06-16	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - L-Alanine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)	splash10-014i-0900000000-381ddf4d9ea77be0b8a5	2014-06-16	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - L-Alanine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)	splash10-01b9-6900000000-6a7c1bb2915e5dd0791f	2014-06-16	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - L-Alanine GC-MS (2 TMS)	splash10-014i-1900000000-84b389f82562c29a8148	2014-06-16	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - L-Alanine EI-B (Non-derivatized)	splash10-00kf-9000000000-b2f7507be509a85d821b	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - L-Alanine EI-B (Non-derivatized)	splash10-014i-0900000000-941672891ab94cf5015d	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - L-Alanine EI-B (Non-derivatized)	splash10-0f79-0910000000-47bd3e3aa274a653a64e	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - L-Alanine GC-EI-TOF (Non-derivatized)	splash10-014i-0900000000-c7f6dbace291e8305a4e	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - L-Alanine GC-EI-TOF (Non-derivatized)	splash10-014i-0900000000-381ddf4d9ea77be0b8a5	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - L-Alanine GC-EI-QQ (Non-derivatized)	splash10-0a4i-1940000000-5def9f7c902aaf1ef607	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - L-Alanine GC-EI-TOF (Non-derivatized)	splash10-01b9-6900000000-6a7c1bb2915e5dd0791f	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - L-Alanine GC-MS (Non-derivatized)	splash10-014i-1900000000-84b389f82562c29a8148	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - L-Alanine GC-MS (Non-derivatized) - 70eV, Positive	splash10-0006-9000000000-d31f7a2ed8284a740b59	2016-09-22	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - L-Alanine GC-MS (1 TMS) - 70eV, Positive	splash10-0006-9100000000-ab365202f52df8e6d401	2017-10-06	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - L-Alanine GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	Wishart Lab	View Spectrum

NMR Spectra

Spectrum Type	Description	Deposition Date	Source	View
Predicted 1D NMR	¹³C NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Experimental 2D NMR	[¹H, ¹H]-TOCSY 2D NMR Spectrum (experimental)	2012-12-04	Wishart Lab	View Spectrum
Experimental 2D NMR	[¹H, ¹³C]-HSQC NMR Spectrum (2D, 600 MHz, H₂O, experimental)	2012-12-05	Wishart Lab	View Spectrum

Biological Properties

Cellular Locations

Cytoplasm
Extracellular
Mitochondria
Lysosome
Peroxisome

Biospecimen Locations

Blood
Breast Milk
Cerebrospinal Fluid (CSF)
Feces
Saliva
Sweat
Urine

Tissue Locations

All Tissues
Placenta
Prostate

Pathways

Name	SMPDB/PathBank	KEGG
Transcription/Translation	SMP00019	Not Available
Urea Cycle
Glucose-Alanine Cycle		Not Available
Selenocompound metabolism	Not Available
Alanine, aspartate and glutamate metabolism	Not Available

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	97-455 uM	Newborn (0-30 days old)	Both	Normal	BC Children's Hos...	details
Blood	Detected and Quantified	134-416 uM	Infant (1 - 3 months old)	Both	Normal	BC Children's Hos...	details
Blood	Detected and Quantified	148-475 uM	Children (3 months - 6 years old)	Both	Normal	BC Children's Hos...	details
Blood	Detected and Quantified	193-545 uM	Children (6 - 18 years old)	Both	Normal	BC Children's Hos...	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	22800120	details
Blood	Detected and Quantified	134-502 uM	Infant (0-1 year old)	Not Specified	Normal	24341803	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	32966057	details
Blood	Detected and Quantified	333.0 (259.0-407.0) uM	Adult (>18 years old)	Both	Normal	12297216	details
Blood	Detected and Quantified	<414 uM	Newborn (0-30 days old)	Not Specified	Normal	26081641	details
Blood	Detected and Quantified	174-375 uM	Children (1-13 years old)	Not Specified	Normal	28649521	details
Blood	Detected and Quantified	240-600 uM	Children (1-13 years old)	Not Specified	Normal	25691415	details
Blood	Detected and Quantified	320 +/- 87 uM	Adult (>18 years old)	Male	Normal	2415198	details
Blood	Detected and Quantified	140 - 460 uM	Children (1 - 13 years old)	Not Specified	Normal	1481808	details
Blood	Detected and Quantified	254 +/- 78 uM	Children (1 - 13 years old)	Female	Normal	2587127	details
Blood	Detected and Quantified	255 +/- 78 uM	Infant (0-1 year old)	Both	Normal	7623444	details
Blood	Detected and Quantified	375.0 +/- 50.0 uM	Newborn (0-30 days old)	Not Specified	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	369.0 +/- 88.0 uM	Children (1-13 years old)	Female	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	444.61(116.67) uM	Adult (>18 years old)	Both	Normal	26010610	details
Blood	Detected and Quantified	448.89(115.71) uM	Adult (>18 years old)	Both	Normal	26010610	details
Blood	Detected and Quantified	200.0-580.0 uM	Adult (>18 years old)	Both	Normal	Molecular You	details
Blood	Detected and Quantified	319.4 +/- 71.7 uM	Adult (>18 years old)	Both	Normal	28278231	details
Blood	Detected and Quantified	465.7(395.5-592.1) uM	Children (1-13 uears old)	Both	Normal	27294788	details
Blood	Detected and Quantified	<500 uM	Infant (0-1 year old)	Not Specified	Normal	172850	details
Blood	Detected and Quantified	427.2 +/- 84.4 uM	Adult (>18 years old)	Both	Normal	21359215	details
Blood	Detected and Quantified	585.0 +/- 22.0 uM	Adult (>18 years old)	Both	Normal	16789974	details
Breast Milk	Detected and Quantified	231 +/- 67.2 uM	Adult (>18 years old)	Female	Normal	24027187	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	46 +/- 27 uM	Adult (>18 years old)	Both	Normal	18502700	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	37.8 +/- 6.8 uM	Adult (>18 years old)	Not Specified	Normal	15911239	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	30.8 +/- 8.7 uM	Adult (>18 years old)	Not Specified	Normal	6198473	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	26.3 +/- 7.4 uM	Adult (>18 years old)	Not Specified	Normal	6198473	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	6-47 uM	Children (1-13 years old)	Not Specified	Normal	24341803	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	23.0-39.5 uM	Children (1-13 years old)	Not Specified	Normal	27912044	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	28.5-38.0 uM	Newborn (0-30 days old)	Not Specified	Normal	27912044	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	27.0 (10.0-44.0) uM	Adult (>18 years old)	Both	Normal	7729054	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	4.23 +/- 0.64 uM	Adult (>18 years old)	Both	Normal	7595563	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	11-53.5 uM	Children (0 - 10 years old)	Both	Normal	BC Children's Hos...	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	10.5-49.5 uM	Adolescent (>11 years old)	Both	Normal	BC Children's Hos...	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	23.2 +/- 5.1 uM	Adult (>18 years old)	Both	Normal	Geigy Scientific ...	details
Feces	Detected but not Quantified	Not Quantified	Children (1-13 years old)	Both	Normal	24130822	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	Normal	Mette S. Sch...	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	21761941	details
Feces	Detected but not Quantified	Not Quantified	Not Specified	Both	Normal	25486321	details
Feces	Detected but not Quantified	Not Quantified	Not Specified	Not Specified	Normal	19573517	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	24029555	details
Feces	Detected but not Quantified	Not Quantified	Not Specified	Not Specified	Normal	22944170	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	25037050	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	27543620	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Not Specified	Normal	25985090	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	18052033	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Both	Normal	27609529	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Normal	27609529	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	Normal	Christopher ...	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	21330412	details
Feces	Detected and Quantified	137.272 +/- 93.0486 nmol/g wet feces	Not Specified	Not Specified	Normal	19573517	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	Normal	26709303	details
Feces	Detected and Quantified	4451.518 +/- 2351.470 nmol/g wet feces	Not Specified	Not Specified	Normal	20669995	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	27065191	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	28842642	details
Feces	Detected and Quantified	2660 +/- 1870 nmol/g wet feces	Adult (>18 years old)	Both	Normal	27624970	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	19006102	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Not Specified	Normal	27622378	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	17269711	details
Feces	Detected and Quantified	600 +/- 470 nmol/g wet feces	Adult (>18 years old)	Both	Normal	27624970	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	27107423	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	25598765	details
Feces	Detected and Quantified	11283 nmol/g wet feces	Adult (>18 years old)	Not Specified	Normal	26416813	details
Feces	Detected but not Quantified	Not Quantified	Not Specified	Not Specified	Normal	20669995	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	29808030	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	23940645	details
Feces	Detected and Quantified	8791 nmol/g wet feces	Adult (>18 years old)	Not Specified	Normal	26416813	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	18085514	details
Feces	Detected and Quantified	12245 nmol/g wet feces	Adult (>18 years old)	Not Specified	Normal	26416813	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	28270806	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	21190195	details
Saliva	Detected and Quantified	17.0 +/- 9.27 uM	Adult (>18 years old)	Both	Normal	Sugimoto et al. (...	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected and Quantified	18.8 +/- 20.4 uM	Adult (>18 years old)	Female	Normal	Sugimoto et al. (...	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected and Quantified	17.4 +/- 4.9 uM	Adult (>18 years old)	Male	Normal	21164041	details
Saliva	Detected and Quantified	14.0 +/- 3.1 uM	Adult (>18 years old)	Male	Normal	21164041	details
Saliva	Detected and Quantified	1-263 uM	Adult (>18 years old)	Male	normal	19259987	details
Saliva	Detected and Quantified	1-65 uM	Adult (>18 years old)	Male	normal	19259987	details
Saliva	Detected and Quantified	20.7 +/- 6.4 uM	Adult (>18 years old)	Male	Normal	21164041	details
Saliva	Detected and Quantified	>10 uM	Adult (>18 years old)	Both	Normal	12097436	details
Saliva	Detected and Quantified	145.27 +/- 108.41 uM	Adult (>18 years old)	Both	Normal	Zerihun T. Dame, ...	details
Saliva	Detected and Quantified	19.2 +/- 5.6 uM	Adult (>18 years old)	Male	Normal	21164041	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	20300169	details
Saliva	Detected and Quantified	57.54 +/- 42.59 uM	Adult (>18 years old)	Female	Normal	23857558	details
Saliva	Detected and Quantified	30.5 +/- 39.6 uM	Adult (>18 years old)	Female	Normal	Sugimoto et al. (...	details
Saliva	Detected and Quantified	26 (7-137) uM	Adult (>18 years old)	Male	Normal	19259987	details
Saliva	Detected and Quantified	23 (4-116) uM	Adult (>18 years old)	Male	Normal	19259987	details
Saliva	Detected and Quantified	27 (<1-96) uM	Adult (>18 years old)	Male	Normal	19259987	details
Saliva	Detected and Quantified	25 (<1-81) uM	Adult (>18 years old)	Female	Normal	19259987	details
Sweat	Detected and Quantified	270-590 uM	Adult (60 years old)	Male	Normal	22194922	details
Sweat	Detected and Quantified	180 uM	Adult (40 years old)	Male	Normal	22194922	details
Sweat	Detected but not Quantified	Not Quantified	Adult	Both	Normal	26755145	details
Urine	Detected and Quantified	4.98-92.078 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	39.0 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	19309105	details
Urine	Detected and Quantified	15.7 +/- 14.7 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	31775291	details
Urine	Detected and Quantified	5.77-78.73 umol/mmol creatinine	Adolescent (13-18 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	9-19 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	27012787	details
Urine	Detected and Quantified	16.84-56.15 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	David F. Putnam C...	details
Urine	Detected and Quantified	6.33-58.60 umol/mmol creatinine	Adult (>18 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	18.72 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	Shaykhutdinov RA,...	details
Urine	Detected and Quantified	152.67 (69.78-235.56) umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	Geigy Scientific ... West Cadwell, N.J... Basel, Switzerlan...	details
Urine	Detected and Quantified	12.3 +/- 5.6 umol/mmol creatinine	Children (1-13 years old)	Male	Normal	Geigy Scientific ... West Cadwell, N.J... Basel, Switzerlan...	details
Urine	Detected and Quantified	22.0 +/- 11.0 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	Geigy Scientific ... West Cadwell, N.J... Basel, Switzerlan...	details
Urine	Detected and Quantified	22.0 +/- 10.0 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	Geigy Scientific ... West Cadwell, N.J... Basel, Switzerlan...	details
Urine	Detected and Quantified	10.52-340.09 umol/mmol creatinine	Infant (0-1 year old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	21.8 (7.1-43.1) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	172.585-558.364 umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	12376931	details
Urine	Detected and Quantified	65.012 +/- 27.635 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Mordechai, Hien, ...	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	33.5 +/- 14.0 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	17474139	details
Urine	Detected and Quantified	34.3 +/- 11.5 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	17474139	details
Urine	Detected and Quantified	8.1 +/- 2.0 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	3168222	details
Urine	Detected and Quantified	8.5 +/- 2.8 umol/mmol creatinine	Infant (6 months - <1 year old)	Both	Normal	3168222	details
Urine	Detected and Quantified	8.8 +/- 2.2 umol/mmol creatinine	Infant (1 - 6 months old)	Both	Normal	3168222	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	16939360	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	16939360	details
Urine	Detected and Quantified	19.46 +/- 5.32 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	15992788	details
Urine	Detected and Quantified	11.42-169.68 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	10-20 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	27012787	details
Urine	Detected and Quantified	21.7 (7.2-47.4) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	85.59 - 284.69 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	81.97 - 239.91 umol/mmol creatinine	Infant (1 - 6 months old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	39.57 - 187.91 umol/mmol creatinine	Infant (6 months - <1 year old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	45.45 - 150.26 umol/mmol creatinine	Children (1 - 2 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	35.95 - 132.73 umol/mmol creatinine	Children (2 - 4 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	16.96 - 105.48 umol/mmol creatinine	Children (4 - 13 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	16.05 - 77.33 umol/mmol creatinine	Adolescent (13 - 21 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	16.05 - 77.33 umol/mmol creatinine	Adult (>21 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	73.1768 +/- 22.3291 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Mordechai, Hien, ...	details
Urine	Detected and Quantified	40.8 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	21388201	details
Urine	Detected and Quantified	68-334 umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	30830347	details
Urine	Detected and Quantified	14-133 umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	30830347	details
Urine	Detected and Quantified	7.24-146.94 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	41-130 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	24341803	details
Urine	Detected and Quantified	260.04 +/- 90.45 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	19468821	details
Urine	Detected and Quantified	395.71 +/- 146.98 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	19468821	details
Urine	Detected and Quantified	610.52 +/- 226.12 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	19468821	details
Urine	Detected and Quantified	113.35 +/- 69.81 umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	2026685	details
Urine	Detected and Quantified	13.2 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	9625050	details
Urine	Detected and Quantified	27-350 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	31506554	details
Urine	Detected and Quantified	32.25-147.8 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	López Hernández Y...	details
Urine	Detected and Quantified	70.70 +/- 24.41 umol/mmol creatinine	Newborn (0-30 days old)	Female	Normal	López Hernández Y...	details
Urine	Detected and Quantified	79.85 +/- 30.57 umol/mmol creatinine	Newborn (0-30 days old)	Male	Normal	López Hernández Y...	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	32966057	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	340 +/- 126.2 uM	Adult (>18 years old)	Both	Heart Transplant	21359215	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Schizophrenia	22800120	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	25105552	details
Blood	Detected and Quantified	293.4 +/- 83.44 uM	Adult (>18 years old)	Female	Pregnancy with fetuses with trisomy 18	23535240	details
Blood	Detected and Quantified	258.62 +/- 85.38 uM	Adult (>18 years old)	Female	Pregnancy	23535240	details
Blood	Detected and Quantified	263.8 (51.5) uM	Adult (>18 years old)	Female	Early preeclampsia	22494326	details
Blood	Detected and Quantified	332.1 (184.2) uM	Adult (>18 years old)	Female	Pregnancy	22494326	details
Blood	Detected and Quantified	455 (221-1086) uM	Children (1-13 years old)	Male	Lipoyltransferase 1 Deficiency	24341803	details
Blood	Detected and Quantified	676 +/- 386 (156-1503) uM	Children (1-13 years old)	Female	Pearson Syndrome	25691415	details
Blood	Detected and Quantified	1088 uM	Newborn (0-30 days old)	Male	Coenzyme Q10 deficiency, primary, 5	26081641	details
Blood	Detected and Quantified	274.0 (250.0-298.0) uM	Adult (>18 years old)	Both	Dengue fever	11237944	details
Blood	Detected and Quantified	958 uM	Infant (0-1 year old)	Not Available	Pyruvate carboxylase deficiency	28649521	details
Blood	Detected and Quantified	296.0 (277.0-316.0) uM	Adult (>18 years old)	Both	Epilepsy	14992292	details
Blood	Detected and Quantified	233-331 uM	Newborn (0-30 days old)	Female	Mitochondrial pyruvate carrier deficiency	12649063	details
Blood	Detected and Quantified	658-1082 uM	Children (1-13 years old)	Both	Pearson Syndrome	25691415	details
Blood	Detected and Quantified	1559 uM	Infant (0-1 year old)	Female	Pearson Syndrome	25691415	details
Blood	Detected and Quantified	707.125 uM	Infant (0-1 year old)	Male	Dihydrolipoamide Dehydrogenase Deficiency	6688766	details
Blood	Detected and Quantified	298 uM	Children (1-13 years old)	Not Available	Pyruvate carboxylase deficiency	28649521	details
Blood	Detected and Quantified	392 +/- 111 uM	Adult (>18 years old)	Male	Schizophrenia	2415198	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	colorectal cancer	21773981	details
Blood	Detected and Quantified	284.62 (65.96) uM	Adult (>18 years old)	Female	Pregnancy with fetus having congenital heart defect	24704061	details
Blood	Detected and Quantified	520 uM	Children (1 - 13 years old)	Female	Leigh's syndrome	2587127	details
Blood	Detected and Quantified	267.32 (53.39) uM	Adult (>18 years old)	Female	Pregnancy	24704061	details
Blood	Detected and Quantified	185-1264 uM	Newborn (0-30 days old)	Male	N-Acetylglutamate synthetase deficiency	7623444	details
Blood	Detected and Quantified	249 +/- 94 uM	Children (1 - 13 years old)	Male	Histidinuria	1481808	details
Blood	Detected and Quantified	261-432 uM	Infant (0-1 year old)	Male	N-Acetylglutamate synthetase deficiency	7623444	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	20156336	details
Blood	Detected and Quantified	31 +/- 8.7 uM	Infant (0-1 year old)	Male	Glucagon deficiency	904979	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Pancreatic cancer	22613268	details
Blood	Detected and Quantified	492.6(418.6-580.2) uM	Children (1-13 uears old)	Both	Environmental enteric dysfunction	27294788	details
Blood	Detected and Quantified	366.8 (204.8) uM	Adult (>18 years old)	Female	Late-onset preeclampsia	23159745	details
Blood	Detected and Quantified	496.62(101.29) uM	Adult (>18 years old)	Both	Heart failure with preserved ejection fraction	26010610	details
Blood	Detected and Quantified	323.8 (151.2) uM	Adult (>18 years old)	Female	Pregnancy	23159745	details
Blood	Detected and Quantified	1000 uM	Infant (0-1 year old)	Male	Pyruvate dehydrogenase phosphatase deficiency	172850	details
Blood	Detected and Quantified	589.0 +/- 15.0 uM	Adult (>18 years old)	Both	Heart failure	16789974	details
Blood	Detected and Quantified	247.2 (89.1) uM	Adult (>18 years old)	Female	Down syndrome pregnancy	23313728	details
Blood	Detected and Quantified	231.9 (87.3) uM	Adult (>18 years old)	Female	Pregnancy	23313728	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	46.1 +/- 13.5 uM	Children (1-13 years old)	Not Specified	Leukemia	15911239	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	34.8 +/- 7.8 uM	Children (1-13 years old)	Not Specified	Leukemia	15911239	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	52 uM	Children (1-13 years old)	Male	Lipoyltransferase 1 Deficiency	24341803	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	48.6 uM	Children (1-13 years old)	Female	Epilepsy, early-onset, vitamin B6-dependent	27912044	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	48.6 uM	Newborn (0-30 days old)	Female	Epilepsy, early-onset, vitamin B6-dependent	27912044	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	0.16 (0.13-0.18) uM	Adult (>18 years old)	Both	Meningitis	7729054	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	19.2 (16.1-22.3) uM	Adult (>18 years old)	Both	Tuberculosis	7729054	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	4.04 +/- 0.57 uM	Adult (>18 years old)	Both	Schizophrenia	7595563	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal Cancer	27275383	details
Feces	Detected but not Quantified	Not Quantified	Children (1-13 years old)	Both	Autism	24130822	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Irritable bowel syndrome	21761941	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Ulcerative colitis	21761941	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	27015276	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Immunoglobulin A nephropathy (IgAN) progressor	26476344	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	ankylosing spondylitis	Tie-juan Sha...	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	rheumatoid arthritis	Tie-juan Sha...	details
Feces	Detected but not Quantified	Not Quantified	Not Specified	Not Specified	Cryptosporidium infection	22944170	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Both	Crohns disease	27609529	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Both	Ulcerative colitis	27609529	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Crohns disease	27609529	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Ulcerative colitis	27609529	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Unclassified IBD	27609529	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	Myalgic encephalomyelitis/chronic fatigue syndrome	Christopher ...	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Crohn disease	28842642	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Irritable bowel syndrome	21330412	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	25105552	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	19006102	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Irritable Bowel Syndrome	21494186	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Ulcerative colitis	28842642	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Not Specified	asymptomatic diverticulosis	27622378	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Not Specified	symptomatic uncomplicated diverticular disease	27622378	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Crohn’s Disease	17269711	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Ulcerative Colitis	17269711	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal Cancer	27107423	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Crohn's disease	17269711	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	25037050	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Crohns disease	25598765	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Ulcerative colitis	25598765	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	23940645	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	23940645	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Gout	28270806	details
Saliva	Detected and Quantified	40.50 +/- 13.83 uM	Adult (>18 years old)	Male	Alzheimer's disease	23857558	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Oral squamous cell carcinoma (OSCC)	21190195	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Oral leukoplakia (OLK)	21190195	details
Saliva	Detected and Quantified	36.63 +/- 16.68 uM	Adult (>18 years old)	Male	Frontotemporal lobe dementia	23857558	details
Saliva	Detected and Quantified	33.94 +/- 19.76 uM	Adult (>18 years old)	Both	Lewy body disease	23857558	details
Saliva	Detected and Quantified	23.0 +/- 8.42 uM	Adult (>18 years old)	Both	Temporomandibular joint disorder (TMD)	Sugimoto et al. (...	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Oral cancer	20300169	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	Breast cancer	20300169	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Not Specified	Pancreatic cancer	20300169	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Not Specified	Periodontal diseases	20300169	details
Urine	Detected and Quantified	427.656-681.457 umol/mmol creatinine	Infant (0-1 year old)	Both	Amish lethal microcephaly	12376931	details
Urine	Detected and Quantified	23 (10-55) umol/mmol creatinine	Not Specified	Both	Lung cancer	18953024	details
Urine	Detected and Quantified	51 +/- 42.068 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Mordechai, Hien, ...	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	25105552	details
Urine	Detected and Quantified	58.4882 +/- 31.7251 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Mordechai, Hien, ...	details
Urine	Detected and Quantified	63.2791 +/- 72.6382 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Gastroesophageal reflux disease	Mordechai, Hien, ...	details
Urine	Detected and Quantified	239 umol/mmol creatinine	Children (1-13 years old)	Male	Lipoyltransferase 1 Deficiency	24341803	details
Urine	Detected and Quantified	76.3 umol/mmol creatinine	Adult (>18 years old)	Both	Paraquat poisoning	9625050	details

Associated Disorders and Diseases

Disease References

Dengue fever
Klassen P, Furst P, Schulz C, Mazariegos M, Solomons NW: Plasma free amino acid concentrations in healthy Guatemalan adults and in patients with classic dengue. Am J Clin Nutr. 2001 Mar;73(3):647-52. [PubMed:11237944 ]
Epilepsy
Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed:14992292 ]
Heart failure
Norrelund H, Wiggers H, Halbirk M, Frystyk J, Flyvbjerg A, Botker HE, Schmitz O, Jorgensen JO, Christiansen JS, Moller N: Abnormalities of whole body protein turnover, muscle metabolism and levels of metabolic hormones in patients with chronic heart failure. J Intern Med. 2006 Jul;260(1):11-21. [PubMed:16789974 ]
Early preeclampsia
Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomics and first-trimester prediction of early-onset preeclampsia. J Matern Fetal Neonatal Med. 2012 Oct;25(10):1840-7. doi: 10.3109/14767058.2012.680254. Epub 2012 Apr 28. [PubMed:22494326 ]
Pregnancy
Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomics and first-trimester prediction of early-onset preeclampsia. J Matern Fetal Neonatal Med. 2012 Oct;25(10):1840-7. doi: 10.3109/14767058.2012.680254. Epub 2012 Apr 28. [PubMed:22494326 ] Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: First-trimester metabolomic detection of late-onset preeclampsia. Am J Obstet Gynecol. 2013 Jan;208(1):58.e1-7. doi: 10.1016/j.ajog.2012.11.003. Epub 2012 Nov 13. [PubMed:23159745 ] Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomic analysis for first-trimester Down syndrome prediction. Am J Obstet Gynecol. 2013 May;208(5):371.e1-8. doi: 10.1016/j.ajog.2012.12.035. Epub 2013 Jan 8. [PubMed:23313728 ] Bahado-Singh RO, Akolekar R, Chelliah A, Mandal R, Dong E, Kruger M, Wishart DS, Nicolaides K: Metabolomic analysis for first-trimester trisomy 18 detection. Am J Obstet Gynecol. 2013 Jul;209(1):65.e1-9. doi: 10.1016/j.ajog.2013.03.028. Epub 2013 Mar 25. [PubMed:23535240 ] Bahado-Singh RO, Ertl R, Mandal R, Bjorndahl TC, Syngelaki A, Han B, Dong E, Liu PB, Alpay-Savasan Z, Wishart DS, Nicolaides KH: Metabolomic prediction of fetal congenital heart defect in the first trimester. Am J Obstet Gynecol. 2014 Sep;211(3):240.e1-240.e14. doi: 10.1016/j.ajog.2014.03.056. Epub 2014 Apr 1. [PubMed:24704061 ]
Late-onset preeclampsia
Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: First-trimester metabolomic detection of late-onset preeclampsia. Am J Obstet Gynecol. 2013 Jan;208(1):58.e1-7. doi: 10.1016/j.ajog.2012.11.003. Epub 2012 Nov 13. [PubMed:23159745 ]
N-acetylglutamate synthetase deficiency
Guffon N, Vianey-Saban C, Bourgeois J, Rabier D, Colombo JP, Guibaud P: A new neonatal case of N-acetylglutamate synthase deficiency treated by carbamylglutamate. J Inherit Metab Dis. 1995;18(1):61-5. [PubMed:7623444 ]
Histidinemia
Nyhan WL, Hilton S: Histidinuria: defective transport of histidine. Am J Med Genet. 1992 Nov 15;44(5):558-61. [PubMed:1481808 ]
Glucagon deficiency
Vidnes J, Oyasaeter S: Glucagon deficiency causing severe neonatal hypoglycemia in a patient with normal insulin secretion. Pediatr Res. 1977 Sep;11(9 Pt 1):943-9. [PubMed:904979 ]
Dihydrolipoamide Dehydrogenase Deficiency
Kuhara T, Shinka T, Inoue Y, Matsumoto M, Yoshino M, Sakaguchi Y, Matsumoto I: Studies of urinary organic acid profiles of a patient with dihydrolipoyl dehydrogenase deficiency. Clin Chim Acta. 1983 Sep 30;133(2):133-40. [PubMed:6688766 ]
Pyruvate dehydrogenase phosphatase deficiency
Robinson BH, Sherwood WG: Pyruvate dehydrogenase phosphatase deficiency: a cause of congenital chronic lactic acidosis in infancy. Pediatr Res. 1975 Dec;9(12):935-9. doi: 10.1203/00006450-197512000-00015. [PubMed:172850 ]
Pearson Syndrome
Crippa BL, Leon E, Calhoun A, Lowichik A, Pasquali M, Longo N: Biochemical abnormalities in Pearson syndrome. Am J Med Genet A. 2015 Mar;167A(3):621-8. doi: 10.1002/ajmg.a.36939. [PubMed:25691415 ]
Coenzyme Q10 deficiency, primary, 5
Danhauser K, Herebian D, Haack TB, Rodenburg RJ, Strom TM, Meitinger T, Klee D, Mayatepek E, Prokisch H, Distelmaier F: Fatal neonatal encephalopathy and lactic acidosis caused by a homozygous loss-of-function variant in COQ9. Eur J Hum Genet. 2016 Mar;24(3):450-4. doi: 10.1038/ejhg.2015.133. Epub 2015 Jun 17. [PubMed:26081641 ]
Pyruvate carboxylase deficiency
Habarou F, Brassier A, Rio M, Chretien D, Monnot S, Barbier V, Barouki R, Bonnefont JP, Boddaert N, Chadefaux-Vekemans B, Le Moyec L, Bastin J, Ottolenghi C, de Lonlay P: Pyruvate carboxylase deficiency: An underestimated cause of lactic acidosis. Mol Genet Metab Rep. 2014 Nov 28;2:25-31. doi: 10.1016/j.ymgmr.2014.11.001. eCollection 2015 Mar. [PubMed:28649521 ]
Mitochondrial pyruvate carrier deficiency
Brivet M, Garcia-Cazorla A, Lyonnet S, Dumez Y, Nassogne MC, Slama A, Boutron A, Touati G, Legrand A, Saudubray JM: Impaired mitochondrial pyruvate importation in a patient and a fetus at risk. Mol Genet Metab. 2003 Mar;78(3):186-92. [PubMed:12649063 ]
Lipoyltransferase 1 Deficiency
Soreze Y, Boutron A, Habarou F, Barnerias C, Nonnenmacher L, Delpech H, Mamoune A, Chretien D, Hubert L, Bole-Feysot C, Nitschke P, Correia I, Sardet C, Boddaert N, Hamel Y, Delahodde A, Ottolenghi C, de Lonlay P: Mutations in human lipoyltransferase gene LIPT1 cause a Leigh disease with secondary deficiency for pyruvate and alpha-ketoglutarate dehydrogenase. Orphanet J Rare Dis. 2013 Dec 17;8:192. doi: 10.1186/1750-1172-8-192. [PubMed:24341803 ]
Meningitis
Wevers RA, Engelke U, Wendel U, de Jong JG, Gabreels FJ, Heerschap A: Standardized method for high-resolution 1H-NMR of cerebrospinal fluid. Clin Chem. 1995 May;41(5):744-51. [PubMed:7729054 ]
Tuberculosis
Wevers RA, Engelke U, Wendel U, de Jong JG, Gabreels FJ, Heerschap A: Standardized method for high-resolution 1H-NMR of cerebrospinal fluid. Clin Chem. 1995 May;41(5):744-51. [PubMed:7729054 ]
Leukemia
Peng CT, Wu KH, Lan SJ, Tsai JJ, Tsai FJ, Tsai CH: Amino acid concentrations in cerebrospinal fluid in children with acute lymphoblastic leukemia undergoing chemotherapy. Eur J Cancer. 2005 May;41(8):1158-63. Epub 2005 Apr 14. [PubMed:15911239 ]
Schizophrenia
Do KQ, Lauer CJ, Schreiber W, Zollinger M, Gutteck-Amsler U, Cuenod M, Holsboer F: gamma-Glutamylglutamine and taurine concentrations are decreased in the cerebrospinal fluid of drug-naive patients with schizophrenic disorders. J Neurochem. 1995 Dec;65(6):2652-62. [PubMed:7595563 ] Bjerkenstedt L, Edman G, Hagenfeldt L, Sedvall G, Wiesel FA: Plasma amino acids in relation to cerebrospinal fluid monoamine metabolites in schizophrenic patients and healthy controls. Br J Psychiatry. 1985 Sep;147:276-82. [PubMed:2415198 ] Cai HL, Li HD, Yan XZ, Sun B, Zhang Q, Yan M, Zhang WY, Jiang P, Zhu RH, Liu YP, Fang PF, Xu P, Yuan HY, Zhang XH, Hu L, Yang W, Ye HS: Metabolomic analysis of biochemical changes in the plasma and urine of first-episode neuroleptic-naive schizophrenia patients after treatment with risperidone. J Proteome Res. 2012 Aug 3;11(8):4338-50. doi: 10.1021/pr300459d. Epub 2012 Jul 26. [PubMed:22800120 ]
Epilepsy, early-onset, vitamin B6-dependent
Darin N, Reid E, Prunetti L, Samuelsson L, Husain RA, Wilson M, El Yacoubi B, Footitt E, Chong WK, Wilson LC, Prunty H, Pope S, Heales S, Lascelles K, Champion M, Wassmer E, Veggiotti P, de Crecy-Lagard V, Mills PB, Clayton PT: Mutations in PROSC Disrupt Cellular Pyridoxal Phosphate Homeostasis and Cause Vitamin-B6-Dependent Epilepsy. Am J Hum Genet. 2016 Dec 1;99(6):1325-1337. doi: 10.1016/j.ajhg.2016.10.011. [PubMed:27912044 ]
Crohn's disease
Marchesi JR, Holmes E, Khan F, Kochhar S, Scanlan P, Shanahan F, Wilson ID, Wang Y: Rapid and noninvasive metabonomic characterization of inflammatory bowel disease. J Proteome Res. 2007 Feb;6(2):546-51. [PubMed:17269711 ] Bjerrum JT, Wang Y, Hao F, Coskun M, Ludwig C, Gunther U, Nielsen OH: Metabonomics of human fecal extracts characterize ulcerative colitis, Crohn's disease and healthy individuals. Metabolomics. 2015;11:122-133. Epub 2014 Jun 1. [PubMed:25598765 ] Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ] Azario I, Pievani A, Del Priore F, Antolini L, Santi L, Corsi A, Cardinale L, Sawamoto K, Kubaski F, Gentner B, Bernardo ME, Valsecchi MG, Riminucci M, Tomatsu S, Aiuti A, Biondi A, Serafini M: Neonatal umbilical cord blood transplantation halts skeletal disease progression in the murine model of MPS-I. Sci Rep. 2017 Aug 25;7(1):9473. doi: 10.1038/s41598-017-09958-9. [PubMed:28842642 ]
Ulcerative colitis
Marchesi JR, Holmes E, Khan F, Kochhar S, Scanlan P, Shanahan F, Wilson ID, Wang Y: Rapid and noninvasive metabonomic characterization of inflammatory bowel disease. J Proteome Res. 2007 Feb;6(2):546-51. [PubMed:17269711 ] Le Gall G, Noor SO, Ridgway K, Scovell L, Jamieson C, Johnson IT, Colquhoun IJ, Kemsley EK, Narbad A: Metabolomics of fecal extracts detects altered metabolic activity of gut microbiota in ulcerative colitis and irritable bowel syndrome. J Proteome Res. 2011 Sep 2;10(9):4208-18. doi: 10.1021/pr2003598. Epub 2011 Aug 8. [PubMed:21761941 ] Bjerrum JT, Wang Y, Hao F, Coskun M, Ludwig C, Gunther U, Nielsen OH: Metabonomics of human fecal extracts characterize ulcerative colitis, Crohn's disease and healthy individuals. Metabolomics. 2015;11:122-133. Epub 2014 Jun 1. [PubMed:25598765 ] Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ] Azario I, Pievani A, Del Priore F, Antolini L, Santi L, Corsi A, Cardinale L, Sawamoto K, Kubaski F, Gentner B, Bernardo ME, Valsecchi MG, Riminucci M, Tomatsu S, Aiuti A, Biondi A, Serafini M: Neonatal umbilical cord blood transplantation halts skeletal disease progression in the murine model of MPS-I. Sci Rep. 2017 Aug 25;7(1):9473. doi: 10.1038/s41598-017-09958-9. [PubMed:28842642 ]
Colorectal cancer
Monleon D, Morales JM, Barrasa A, Lopez JA, Vazquez C, Celda B: Metabolite profiling of fecal water extracts from human colorectal cancer. NMR Biomed. 2009 Apr;22(3):342-8. doi: 10.1002/nbm.1345. [PubMed:19006102 ] Weir TL, Manter DK, Sheflin AM, Barnett BA, Heuberger AL, Ryan EP: Stool microbiome and metabolome differences between colorectal cancer patients and healthy adults. PLoS One. 2013 Aug 6;8(8):e70803. doi: 10.1371/journal.pone.0070803. Print 2013. [PubMed:23940645 ] Ritchie SA, Ahiahonu PW, Jayasinghe D, Heath D, Liu J, Lu Y, Jin W, Kavianpour A, Yamazaki Y, Khan AM, Hossain M, Su-Myat KK, Wood PL, Krenitsky K, Takemasa I, Miyake M, Sekimoto M, Monden M, Matsubara H, Nomura F, Goodenowe DB: Reduced levels of hydroxylated, polyunsaturated ultra long-chain fatty acids in the serum of colorectal cancer patients: implications for early screening and detection. BMC Med. 2010 Feb 15;8:13. doi: 10.1186/1741-7015-8-13. [PubMed:20156336 ] Ni Y, Xie G, Jia W: Metabonomics of human colorectal cancer: new approaches for early diagnosis and biomarker discovery. J Proteome Res. 2014 Sep 5;13(9):3857-70. doi: 10.1021/pr500443c. Epub 2014 Aug 14. [PubMed:25105552 ] Ikeda A, Nishiumi S, Shinohara M, Yoshie T, Hatano N, Okuno T, Bamba T, Fukusaki E, Takenawa T, Azuma T, Yoshida M: Serum metabolomics as a novel diagnostic approach for gastrointestinal cancer. Biomed Chromatogr. 2012 May;26(5):548-58. doi: 10.1002/bmc.1671. Epub 2011 Jul 20. [PubMed:21773981 ] Lin Y, Ma C, Liu C, Wang Z, Yang J, Liu X, Shen Z, Wu R: NMR-based fecal metabolomics fingerprinting as predictors of earlier diagnosis in patients with colorectal cancer. Oncotarget. 2016 May 17;7(20):29454-64. doi: 10.18632/oncotarget.8762. [PubMed:27107423 ] Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016. [PubMed:27275383 ] Sinha R, Ahn J, Sampson JN, Shi J, Yu G, Xiong X, Hayes RB, Goedert JJ: Fecal Microbiota, Fecal Metabolome, and Colorectal Cancer Interrelations. PLoS One. 2016 Mar 25;11(3):e0152126. doi: 10.1371/journal.pone.0152126. eCollection 2016. [PubMed:27015276 ] Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050 ]
Irritable bowel syndrome
Ponnusamy K, Choi JN, Kim J, Lee SY, Lee CH: Microbial community and metabolomic comparison of irritable bowel syndrome faeces. J Med Microbiol. 2011 Jun;60(Pt 6):817-27. doi: 10.1099/jmm.0.028126-0. Epub 2011 Feb 17. [PubMed:21330412 ] Le Gall G, Noor SO, Ridgway K, Scovell L, Jamieson C, Johnson IT, Colquhoun IJ, Kemsley EK, Narbad A: Metabolomics of fecal extracts detects altered metabolic activity of gut microbiota in ulcerative colitis and irritable bowel syndrome. J Proteome Res. 2011 Sep 2;10(9):4208-18. doi: 10.1021/pr2003598. Epub 2011 Aug 8. [PubMed:21761941 ] Hong YS, Hong KS, Park MH, Ahn YT, Lee JH, Huh CS, Lee J, Kim IK, Hwang GS, Kim JS: Metabonomic understanding of probiotic effects in humans with irritable bowel syndrome. J Clin Gastroenterol. 2011 May-Jun;45(5):415-25. doi: 10.1097/MCG.0b013e318207f76c. [PubMed:21494186 ]
Autism
De Angelis M, Piccolo M, Vannini L, Siragusa S, De Giacomo A, Serrazzanetti DI, Cristofori F, Guerzoni ME, Gobbetti M, Francavilla R: Fecal microbiota and metabolome of children with autism and pervasive developmental disorder not otherwise specified. PLoS One. 2013 Oct 9;8(10):e76993. doi: 10.1371/journal.pone.0076993. eCollection 2013. [PubMed:24130822 ]
Diverticular disease
Tursi A, Mastromarino P, Capobianco D, Elisei W, Miccheli A, Capuani G, Tomassini A, Campagna G, Picchio M, Giorgetti G, Fabiocchi F, Brandimarte G: Assessment of Fecal Microbiota and Fecal Metabolome in Symptomatic Uncomplicated Diverticular Disease of the Colon. J Clin Gastroenterol. 2016 Oct;50 Suppl 1:S9-S12. doi: 10.1097/MCG.0000000000000626. [PubMed:27622378 ]
Gout
Shao T, Shao L, Li H, Xie Z, He Z, Wen C: Combined Signature of the Fecal Microbiome and Metabolome in Patients with Gout. Front Microbiol. 2017 Feb 21;8:268. doi: 10.3389/fmicb.2017.00268. eCollection 2017. [PubMed:28270806 ]
Rheumatoid arthritis
Tie-juan ShaoZhi-xing HeZhi-jun XieHai-chang LiMei-jiao WangCheng-ping Wen. Characterization of ankylosing spondylitis and rheumatoid arthritis using 1H NMR-based metabolomics of human fecal extracts. Metabolomics. April 2016, 12:70 [Link]
Perillyl alcohol administration for cancer treatment
Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ]
Pancreatic cancer
Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ] Zhang L, Jin H, Guo X, Yang Z, Zhao L, Tang S, Mo P, Wu K, Nie Y, Pan Y, Fan D: Distinguishing pancreatic cancer from chronic pancreatitis and healthy individuals by (1)H nuclear magnetic resonance-based metabonomic profiles. Clin Biochem. 2012 Sep;45(13-14):1064-9. doi: 10.1016/j.clinbiochem.2012.05.012. Epub 2012 May 19. [PubMed:22613268 ]
Periodontal disease
Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ]
Alzheimer's disease
Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Frontotemporal dementia
Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Lewy body disease
Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Temporomandibular joint disorder
(). Sugimoto et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles. . .
Paraquat poisoning
Bairaktari E, Katopodis K, Siamopoulos KC, Tsolas O: Paraquat-induced renal injury studied by 1H nuclear magnetic resonance spectroscopy of urine. Clin Chem. 1998 Jun;44(6 Pt 1):1256-61. [PubMed:9625050 ]
Lung Cancer
Wishart DS, Knox C, Guo AC, Eisner R, Young N, Gautam B, Hau DD, Psychogios N, Dong E, Bouatra S, Mandal R, Sinelnikov I, Xia J, Jia L, Cruz JA, Lim E, Sobsey CA, Shrivastava S, Huang P, Liu P, Fang L, Peng J, Fradette R, Cheng D, Tzur D, Clements M, Lewis A, De Souza A, Zuniga A, Dawe M, Xiong Y, Clive D, Greiner R, Nazyrova A, Shaykhutdinov R, Li L, Vogel HJ, Forsythe I: HMDB: a knowledgebase for the human metabolome. Nucleic Acids Res. 2009 Jan;37(Database issue):D603-10. doi: 10.1093/nar/gkn810. Epub 2008 Oct 25. [PubMed:18953024 ]
Eosinophilic esophagitis
(). Mordechai, Hien, and David S. Wishart. .
Amish lethal microcephaly
Kelley RI, Robinson D, Puffenberger EG, Strauss KA, Morton DH: Amish lethal microcephaly: a new metabolic disorder with severe congenital microcephaly and 2-ketoglutaric aciduria. Am J Med Genet. 2002 Nov 1;112(4):318-26. doi: 10.1002/ajmg.10529. [PubMed:12376931 ]

Associated OMIM IDs

608300 (N-acetylglutamate synthetase deficiency)
235800 (Histidinemia)
246900 (Dihydrolipoamide Dehydrogenase Deficiency)
608782 (Pyruvate dehydrogenase phosphatase deficiency)
557000 (Pearson Syndrome)
614654 (Coenzyme Q10 deficiency, primary, 5)
266150 (Pyruvate carboxylase deficiency)
614741 (Mitochondrial pyruvate carrier deficiency)
616299 (Lipoyltransferase 1 Deficiency)
607948 (Tuberculosis)
181500 (Schizophrenia)
617290 (Epilepsy, early-onset, vitamin B6-dependent)
266600 (Crohn's disease)
114500 (Colorectal cancer)
209850 (Autism)
138900 (Gout)
180300 (Rheumatoid arthritis)
260350 (Pancreatic cancer)
170650 (Periodontal disease)
104300 (Alzheimer's disease)
600274 (Frontotemporal dementia)
211980 (Lung Cancer)
610247 (Eosinophilic esophagitis)
607196 (Amish lethal microcephaly)

External Links

DrugBank ID

DB00160

Phenol Explorer Compound ID

Not Available

FooDB ID

KNApSAcK ID

Chemspider ID

KEGG Compound ID

BioCyc ID

BiGG ID

Wikipedia Link

METLIN ID

Not Available

PubChem Compound

5950

PDB ID

Not Available

ChEBI ID

16977

Food Biomarker Ontology

Not Available

VMH ID

ALA_L

MarkerDB ID

MDB00000078

Good Scents ID

Not Available

References

Synthesis Reference

Chibata I; Kakimoto T; Kato J Enzymatic production of L-alanine by Pseudomonas dacunhae. Applied microbiology (1965), 13(5), 638-45.

Material Safety Data Sheet (MSDS)

Download (PDF)

General References

Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. doi: 10.1038/nature07762. [PubMed:19212411 ]
Silwood CJ, Lynch E, Claxson AW, Grootveld MC: 1H and (13)C NMR spectroscopic analysis of human saliva. J Dent Res. 2002 Jun;81(6):422-7. [PubMed:12097436 ]
Nicholson JK, O'Flynn MP, Sadler PJ, Macleod AF, Juul SM, Sonksen PH: Proton-nuclear-magnetic-resonance studies of serum, plasma and urine from fasting normal and diabetic subjects. Biochem J. 1984 Jan 15;217(2):365-75. [PubMed:6696735 ]
Bairaktari E, Katopodis K, Siamopoulos KC, Tsolas O: Paraquat-induced renal injury studied by 1H nuclear magnetic resonance spectroscopy of urine. Clin Chem. 1998 Jun;44(6 Pt 1):1256-61. [PubMed:9625050 ]
Wevers RA, Engelke U, Wendel U, de Jong JG, Gabreels FJ, Heerschap A: Standardized method for high-resolution 1H-NMR of cerebrospinal fluid. Clin Chem. 1995 May;41(5):744-51. [PubMed:7729054 ]
Hagenfeldt L, Bjerkenstedt L, Edman G, Sedvall G, Wiesel FA: Amino acids in plasma and CSF and monoamine metabolites in CSF: interrelationship in healthy subjects. J Neurochem. 1984 Mar;42(3):833-7. [PubMed:6198473 ]
Peng CT, Wu KH, Lan SJ, Tsai JJ, Tsai FJ, Tsai CH: Amino acid concentrations in cerebrospinal fluid in children with acute lymphoblastic leukemia undergoing chemotherapy. Eur J Cancer. 2005 May;41(8):1158-63. Epub 2005 Apr 14. [PubMed:15911239 ]
Cynober LA: Plasma amino acid levels with a note on membrane transport: characteristics, regulation, and metabolic significance. Nutrition. 2002 Sep;18(9):761-6. [PubMed:12297216 ]
Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed:14992292 ]
Klassen P, Furst P, Schulz C, Mazariegos M, Solomons NW: Plasma free amino acid concentrations in healthy Guatemalan adults and in patients with classic dengue. Am J Clin Nutr. 2001 Mar;73(3):647-52. [PubMed:11237944 ]
Iioka H, Hisanaga H, Moriyama IS, Akada S, Shimamoto T, Yamada Y, Ichijo M: Characterization of human placental activity for transport of L-alanine, using brush border (microvillous) membrane vesicles. Placenta. 1992 Mar-Apr;13(2):179-90. [PubMed:1631030 ]
Saklatvala J: Hydrolysis of the elastase substrate succinyltrialanine nitroanilide by a metal-dependent enzyme in rheumatoid synovial fluid. J Clin Invest. 1977 May;59(5):794-801. [PubMed:16038 ]
Stahl A, Frick A, Imler M, Schlienger JL: Semiautomated enzymic microassay for plasma L-alanine. Enzyme. 1979;24(5):294-301. [PubMed:41707 ]
Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043. [PubMed:32033212 ]

Only showing the first 10 proteins. There are 21 proteins in total.

Show all enzymes and transporters

Enzymes

Enzyme Details

1. 4-aminobutyrate aminotransferase, mitochondrial

General function:: Involved in 4-aminobutyrate transaminase activity
Specific function:: Catalyzes the conversion of gamma-aminobutyrate and L-beta-aminoisobutyrate to succinate semialdehyde and methylmalonate semialdehyde, respectively. Can also convert delta-aminovalerate and beta-alanine.
Gene Name:: ABAT
Uniprot ID:: P80404
Molecular weight:: 56438.405

References

Amadasi A, Bertoldi M, Contestabile R, Bettati S, Cellini B, di Salvo ML, Borri-Voltattorni C, Bossa F, Mozzarelli A: Pyridoxal 5'-phosphate enzymes as targets for therapeutic agents. Curr Med Chem. 2007;14(12):1291-324. [PubMed:17504214 ]
Chen X, Ji ZL, Chen YZ: TTD: Therapeutic Target Database. Nucleic Acids Res. 2002 Jan 1;30(1):412-5. [PubMed:11752352 ]

Enzyme Details

2. Kynureninase

General function:: Involved in metabolic process
Specific function:: Catalyzes the cleavage of L-kynurenine (L-Kyn) and L-3-hydroxykynurenine (L-3OHKyn) into anthranilic acid (AA) and 3-hydroxyanthranilic acid (3-OHAA), respectively. Has a preference for the L-3-hydroxy form. Also has cysteine-conjugate-beta-lyase activity.
Gene Name:: KYNU
Uniprot ID:: Q16719
Molecular weight:: 34634.47

Reactions

L-Kynurenine + Water → 2-Aminobenzoic acid + L-Alanine	details
L-3-Hydroxykynurenine + Water → 3-Hydroxyanthranilic acid + L-Alanine	details
L-Formylkynurenine + Water → Formylanthranilic acid + L-Alanine	details

References

Christensen M, Duno M, Lund AM, Skovby F, Christensen E: Xanthurenic aciduria due to a mutation in KYNU encoding kynureninase. J Inherit Metab Dis. 2007 Apr;30(2):248-55. Epub 2007 Mar 1. [PubMed:17334708 ]
Lima S, Khristoforov R, Momany C, Phillips RS: Crystal structure of Homo sapiens kynureninase. Biochemistry. 2007 Mar 13;46(10):2735-44. Epub 2007 Feb 15. [PubMed:17300176 ]

Enzyme Details

3. Alanine--glyoxylate aminotransferase 2, mitochondrial

General function:: Involved in transaminase activity
Specific function:: Can metabolize asymmetric dimethylarginine (ADMA) via transamination to alpha-keto-delta-(NN-dimethylguanidino) valeric acid (DMGV). ADMA is a potent inhibitor of nitric-oxide (NO) synthase, and this activity provides mechanism through which the kidney regulates blood pressure.
Gene Name:: AGXT2
Uniprot ID:: Q9BYV1
Molecular weight:: 57155.905

Reactions

L-Alanine + Glyoxylic acid → Pyruvic acid + Glycine	details
(R)-beta-Aminoisobutyric acid + Pyruvic acid → 2-Methyl-3-oxopropanoic acid + L-Alanine	details

References

Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
Okuno E, Ishikawa T, Kawai J, Kido R: Alanine:glyoxylate aminotransferase activities in liver of Suncus murinus (insectivora). Comp Biochem Physiol B. 1988;90(4):773-8. [PubMed:2907870 ]
Takada Y, Mori T, Noguchi T: The effect of vitamin B6 deficiency on alanine: glyoxylate aminotransferase isoenzymes in rat liver. Arch Biochem Biophys. 1984 Feb 15;229(1):1-6. [PubMed:6703688 ]

Enzyme Details

4. Alanine--tRNA ligase, cytoplasmic

General function:: Involved in nucleotide binding
Specific function:: Catalyzes the attachment of alanine to tRNA(Ala) in a two-step reaction: alanine is first activated by ATP to form Ala-AMP and then transferred to the acceptor end of tRNA(Ala). Also edits incorrectly charged tRNA(Ala) via its editing domain (By similarity).
Gene Name:: AARS
Uniprot ID:: P49588
Molecular weight:: 106809.525

Reactions

Adenosine triphosphate + L-Alanine + tRNA(Ala) → Adenosine monophosphate + Pyrophosphate + L-alanyl-tRNA(Ala)	details
Adenosine triphosphate + L-Alanine + tRNA(Ala) → Adenosine monophosphate + Pyrophosphate + L-Alanyl-tRNA	details

References

Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
Nagan MC, Beuning P, Musier-Forsyth K, Cramer CJ: Importance of discriminator base stacking interactions: molecular dynamics analysis of A73 microhelix(Ala) variants. Nucleic Acids Res. 2000 Jul 1;28(13):2527-34. [PubMed:10871402 ]
McClain WH, Gabriel K, Schneider J: Specific function of a G.U wobble pair from an adjacent helical site in tRNA(Ala) during recognition by alanyl-tRNA synthetase. RNA. 1996 Feb;2(2):105-9. [PubMed:8601277 ]
Buechter DD, Schimmel P: Minor groove recognition of the critical acceptor helix base pair by an appended module of a class II tRNA synthetase. Biochemistry. 1995 May 9;34(18):6014-9. [PubMed:7742303 ]

Enzyme Details

5. Serine--pyruvate aminotransferase

General function:: Involved in metabolic process
Specific function:: Not Available
Gene Name:: AGXT
Uniprot ID:: P21549
Molecular weight:: 43009.535

Reactions

L-Serine + Pyruvic acid → Hydroxypyruvic acid + L-Alanine	details
L-Alanine + Glyoxylic acid → Pyruvic acid + Glycine	details

References

Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
Salido EC, Li XM, Lu Y, Wang X, Santana A, Roy-Chowdhury N, Torres A, Shapiro LJ, Roy-Chowdhury J: Alanine-glyoxylate aminotransferase-deficient mice, a model for primary hyperoxaluria that responds to adenoviral gene transfer. Proc Natl Acad Sci U S A. 2006 Nov 28;103(48):18249-54. Epub 2006 Nov 16. [PubMed:17110443 ]
Amoroso A, Pirulli D, Florian F, Puzzer D, Boniotto M, Crovella S, Zezlina S, Spano A, Mazzola G, Savoldi S, Ferrettini C, Berutti S, Petrarulo M, Marangella M: AGXT gene mutations and their influence on clinical heterogeneity of type 1 primary hyperoxaluria. J Am Soc Nephrol. 2001 Oct;12(10):2072-9. [PubMed:11562405 ]
Pirulli D, Puzzer D, Ferri L, Crovella S, Amoroso A, Ferrettini C, Marangella M, Mazzola G, Florian F: Molecular analysis of hyperoxaluria type 1 in Italian patients reveals eight new mutations in the alanine: glyoxylate aminotransferase gene. Hum Genet. 1999 Jun;104(6):523-5. [PubMed:10453743 ]

Enzyme Details

6. Alanine aminotransferase 1

General function:: Involved in 1-aminocyclopropane-1-carboxylate synthase activity
Specific function:: Catalyzes the reversible transamination between alanine and 2-oxoglutarate to form pyruvate and glutamate. Participates in cellular nitrogen metabolism and also in liver gluconeogenesis starting with precursors transported from skeletal muscles (By similarity).
Gene Name:: GPT
Uniprot ID:: P24298
Molecular weight:: 54636.415

Reactions

L-Alanine + Oxoglutaric acid → Pyruvic acid + L-Glutamic acid	details

References

Miyashita Y, Dolferus R, Ismond KP, Good AG: Alanine aminotransferase catalyses the breakdown of alanine after hypoxia in Arabidopsis thaliana. Plant J. 2007 Mar;49(6):1108-21. Epub 2007 Feb 22. [PubMed:17319845 ]
Gray S, Wang B, Orihuela Y, Hong EG, Fisch S, Haldar S, Cline GW, Kim JK, Peroni OD, Kahn BB, Jain MK: Regulation of gluconeogenesis by Kruppel-like factor 15. Cell Metab. 2007 Apr;5(4):305-12. [PubMed:17403374 ]
Taracha E, Habrat B, Chrapusta SJ, Lehner M, Wislowska A, Woronowicz BT, Bogulas M, Charewicz J, Markuszewski C, Plaznik A: Combining markers of nephrotoxicity and hepatotoxicity for improved monitoring and detection of chronic alcohol abuse. Clin Chem Lab Med. 2006;44(12):1446-52. [PubMed:17163821 ]
Chen CH, Lee RP, Wu WT, Liao KW, Hsu N, Hsu BG: Fluvastatin ameliorates endotoxin induced multiple organ failure in conscious rats. Resuscitation. 2007 Jul;74(1):166-74. Epub 2007 Mar 13. [PubMed:17353078 ]

Enzyme Details

7. Peptidoglycan recognition protein 1

General function:: Involved in N-acetylmuramoyl-L-alanine amidase activity
Specific function:: Pattern receptor that binds to murein peptidoglycans (PGN) of Gram-positive bacteria. Has bactericidal activity towards Gram-positive bacteria. May kill Gram-positive bacteria by interfering with peptidoglycan biosynthesis. Binds also to Gram-negative bacteria, and has bacteriostatic activity towards Gram-negative bacteria. Plays a role in innate immunity.
Gene Name:: PGLYRP1
Uniprot ID:: O75594
Molecular weight:: Not Available

Reactions

N-Acetylmuramoyl-Ala + Water → N-Acetylmuramate + L-Alanine	details

Enzyme Details

8. Cysteine desulfurase, mitochondrial

General function:: Involved in metabolic process
Specific function:: Catalyzes the removal of elemental sulfur from cysteine to produce alanine. It supplies the inorganic sulfur for iron-sulfur (Fe-S) clusters. May be involved in the biosynthesis of molybdenum cofactor.
Gene Name:: NFS1
Uniprot ID:: Q9Y697
Molecular weight:: 44361.485

Reactions

L-Cysteine + acceptor → L-Alanine + S-sulfanyl-acceptor	details
[Enzyme]-cysteine + L-Cysteine → [Enzyme]-S-sulfanylcysteine + L-Alanine	details

References

You D, Wang L, Yao F, Zhou X, Deng Z: A novel DNA modification by sulfur: DndA is a NifS-like cysteine desulfurase capable of assembling DndC as an iron-sulfur cluster protein in Streptomyces lividans. Biochemistry. 2007 May 22;46(20):6126-33. Epub 2007 May 1. [PubMed:17469805 ]
Zeng J, Zhang Y, Liu Y, Zhang X, Xia L, Liu J, Qiu G: Expression, purification and characterization of a cysteine desulfurase, IscS, from Acidithiobacillus ferrooxidans. Biotechnol Lett. 2007 Dec;29(12):1983-90. Epub 2007 Jul 28. [PubMed:17660944 ]

Enzyme Details

9. Neutral amino acid transporter A

General function:: Involved in sodium:dicarboxylate symporter activity
Specific function:: Transporter for alanine, serine, cysteine, and threonine. Exhibits sodium dependence
Gene Name:: SLC1A4
Uniprot ID:: P43007
Molecular weight:: 55722.5

References

Zhang Z, Papageorgiou G, Corrie JE, Grewer C: Pre-steady-state currents in neutral amino acid transporters induced by photolysis of a new caged alanine derivative. Biochemistry. 2007 Mar 27;46(12):3872-80. Epub 2007 Feb 21. [PubMed:17311416 ]
Takasaki C, Miura E, Watanabe M: Segmental and complementary expression of L-serine biosynthetic enzyme 3-phosphoglycerate dehydrogenase and neutral amino acid transporter ASCT1 in the mouse kidney. Biomed Res. 2007 Apr;28(2):61-9. [PubMed:17510490 ]
Zhang Z, Grewer C: The sodium-coupled neutral amino acid transporter SNAT2 mediates an anion leak conductance that is differentially inhibited by transported substrates. Biophys J. 2007 Apr 1;92(7):2621-32. Epub 2007 Jan 19. [PubMed:17237199 ]
Pinho MJ, Pinto V, Serrao MP, Jose PA, Soares-da-Silva P: Underexpression of the Na+-dependent neutral amino acid transporter ASCT2 in the spontaneously hypertensive rat kidney. Am J Physiol Regul Integr Comp Physiol. 2007 Jul;293(1):R538-47. Epub 2007 May 2. [PubMed:17475673 ]
Wu Y, Shen D, Chen Z, Clayton S, Vadgama JV: Taxol induced apoptosis regulates amino acid transport in breast cancer cells. Apoptosis. 2007 Mar;12(3):593-612. Epub 2006 Dec 29. [PubMed:17195090 ]

Enzyme Details

10. Alanine aminotransferase 2

General function:: Involved in transferase activity, transferring nitrogenous groups
Specific function:: Catalyzes the reversible transamination between alanine and 2-oxoglutarate to form pyruvate and glutamate.
Gene Name:: GPT2
Uniprot ID:: Q8TD30
Molecular weight:: 46982.6

Reactions

L-Alanine + Oxoglutaric acid → Pyruvic acid + L-Glutamic acid	details

References

Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
Yang RZ, Blaileanu G, Hansen BC, Shuldiner AR, Gong DW: cDNA cloning, genomic structure, chromosomal mapping, and functional expression of a novel human alanine aminotransferase. Genomics. 2002 Mar;79(3):445-50. [PubMed:11863375 ]
Jadhao SB, Yang RZ, Lin Q, Hu H, Anania FA, Shuldiner AR, Gong DW: Murine alanine aminotransferase: cDNA cloning, functional expression, and differential gene regulation in mouse fatty liver. Hepatology. 2004 May;39(5):1297-302. [PubMed:15122758 ]
Rajamohan F, Nelms L, Joslin DL, Lu B, Reagan WJ, Lawton M: cDNA cloning, expression, purification, distribution, and characterization of biologically active canine alanine aminotransferase-1. Protein Expr Purif. 2006 Jul;48(1):81-9. Epub 2006 Jan 30. [PubMed:16495081 ]

Transporters

Enzyme Details

1. Large neutral amino acids transporter small subunit 2

General function:: Involved in transport
Specific function:: Sodium-independent, high-affinity transport of small and large neutral amino acids such as alanine, serine, threonine, cysteine, phenylalanine, tyrosine, leucine, arginine and tryptophan, when associated with SLC3A2/4F2hc. Acts as an amino acid exchanger. Has higher affinity for L-phenylalanine than LAT1 but lower affinity for glutamine and serine. L-alanine is transported at physiological concentrations. Plays a role in basolateral (re)absorption of neutral amino acids. Involved in the uptake of methylmercury (MeHg) when administered as the L-cysteine or D,L-homocysteine complexes, and hence plays a role in metal ion homeostasis and toxicity. Involved in the cellular activity of small molecular weight nitrosothiols, via the stereoselective transport of L-nitrosocysteine (L-CNSO) across the transmembrane. Plays an essential role in the reabsorption of neutral amino acids from the epithelial cells to the bloodstream in the kidney
Gene Name:: SLC7A8
Uniprot ID:: Q9UHI5
Molecular weight:: 58381.1

References

Broer S, Broer A, Hansen JT, Bubb WA, Balcar VJ, Nasrallah FA, Garner B, Rae C: Alanine metabolism, transport, and cycling in the brain. J Neurochem. 2007 Sep;102(6):1758-70. Epub 2007 May 14. [PubMed:17504263 ]

Enzyme Details

2. Sodium-coupled neutral amino acid transporter 3

General function:: Amino acid transport and metabolism
Specific function:: Sodium-dependent amino acid/proton antiporter. Mediates electrogenic cotransport of glutamine and sodium ions in exchange for protons. Also recognizes histidine, asparagine and alanine. May mediate amino acid transport in either direction under physiological conditions. May play a role in nitrogen metabolism and synaptic transmission
Gene Name:: SLC38A3
Uniprot ID:: Q99624
Molecular weight:: 55772.4

Enzyme Details

3. Proton-coupled amino acid transporter 1

General function:: Amino acid transport and metabolism
Specific function:: Neutral amino acid/proton symporter. Has a pH-dependent electrogenic transport activity for small amino acids such as glycine, alanine and proline. Besides small apolar L-amino acids, it also recognize their D-enantiomers and selected amino acid derivatives such as gamma-aminobutyric acid
Gene Name:: SLC36A1
Uniprot ID:: Q7Z2H8
Molecular weight:: 53075.0

References

Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
Miyauchi S, Abbot EL, Zhuang L, Subramanian R, Ganapathy V, Thwaites DT: Isolation and function of the amino acid transporter PAT1 (slc36a1) from rabbit and discrimination between transport via PAT1 and system IMINO in renal brush-border membrane vesicles. Mol Membr Biol. 2005 Nov-Dec;22(6):549-59. [PubMed:16373326 ]

Enzyme Details

4. Monocarboxylate transporter 10

General function:: Involved in transmembrane transport
Specific function:: Sodium-independent transporter that mediates the update of aromatic acid. Can function as a net efflux pathway for aromatic amino acids in the basosolateral epithelial cells
Gene Name:: SLC16A10
Uniprot ID:: Q8TF71
Molecular weight:: 55492.1

References

Kim DK, Kanai Y, Chairoungdua A, Matsuo H, Cha SH, Endou H: Expression cloning of a Na+-independent aromatic amino acid transporter with structural similarity to H+/monocarboxylate transporters. J Biol Chem. 2001 May 18;276(20):17221-8. Epub 2001 Feb 20. [PubMed:11278508 ]

Only showing the first 10 proteins. There are 21 proteins in total.

Show all enzymes and transporters

Showing metabocard for L-Alanine (HMDB0000161)

MOL for HMDB0000161 (L-Alanine)

3D MOL for HMDB0000161 (L-Alanine)

3D SDF for HMDB0000161 (L-Alanine)

3D-SDF for HMDB0000161 (L-Alanine)

PDB for HMDB0000161 (L-Alanine)

3D PDB for HMDB0000161 (L-Alanine)

SMILES for HMDB0000161 (L-Alanine)

INCHI for HMDB0000161 (L-Alanine)

Structure for HMDB0000161 (L-Alanine)

3D Structure for HMDB0000161 (L-Alanine)

Predicted Collision Cross Sections

Predicted Kovats Retention Indices

Derivatized

GC-MS Spectra

NMR Spectra

Enzymes

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Transporters

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