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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2021-09-14 15:46:58 UTC
HMDB IDHMDB0000182
Secondary Accession Numbers
  • HMDB00182
  • HMDB0062809
  • HMDB62809
Metabolite Identification
Common NameL-Lysine
DescriptionLysine (Lys), also known as L-lysine 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 (–NH2) and carboxyl (–COOH) functional groups, along with a side chain (R group) specific to each amino acid. Lysine is one of 20 proteinogenic amino acids, i.e., the amino acids used in the biosynthesis of proteins. Lysine is found in all organisms ranging from bacteria to plants to animals. It is classified as an aliphatic, positively charged or basic amino acid. In humans, lysine is an essential amino acid, meaning the body cannot synthesize it, and it must be obtained from the diet. Lysine is high in foods such as wheat germ, cottage cheese and chicken. Of meat products, wild game and pork have the highest concentration of lysine. Fruits and vegetables contain little lysine, except avocados. Normal requirements for lysine have been found to be about 8 g per day or 12 mg/kg in adults. Children and infants need more, 44 mg/kg per day for an eleven to-twelve-year old, and 97 mg/kg per day for three-to six-month old. In organisms that synthesise lysine, it has two main biosynthetic pathways, the diaminopimelate and α-aminoadipate pathways, which employ distinct enzymes and substrates and are found in diverse organisms. Lysine catabolism occurs through one of several pathways, the most common of which is the saccharopine pathway. Lysine plays several roles in humans, most importantly proteinogenesis, but also in the crosslinking of collagen polypeptides, uptake of essential mineral nutrients, and in the production of carnitine, which is key in fatty acid metabolism. Lysine is also often involved in histone modifications, and thus, impacts the epigenome. Lysine is highly concentrated in muscle compared to most other amino acids. Normal lysine metabolism is dependent upon many nutrients including niacin, vitamin B6, riboflavin, vitamin C, glutamic acid and iron. Excess arginine antagonizes lysine. Several inborn errors of lysine metabolism are known, such as cystinuria, hyperdibasic aminoaciduria I, lysinuric protein intolerance, propionic acidemia, and tyrosinemia I. Most are marked by mental retardation with occasional diverse symptoms such as absence of secondary sex characteristics, undescended testes, abnormal facial structure, anemia, obesity, enlarged liver and spleen, and eye muscle imbalance. Lysine also may be a useful adjunct in the treatment of osteoporosis. Although high protein diets result in loss of large amounts of calcium in urine, so does lysine deficiency. Lysine may be an adjunct therapy because it reduces calcium losses in urine. Lysine deficiency also may result in immunodeficiency. Requirements for lysine are probably increased by stress. Lysine toxicity has not occurred with oral doses in humans. Lysine dosages are presently too small and may fail to reach the concentrations necessary to prove potential therapeutic applications. Lysine metabolites, amino caproic acid and carnitine have already shown their therapeutic potential. Thirty grams daily of amino caproic acid has been used as an initial daily dose in treating blood clotting disorders, indicating that the proper doses of lysine, its precursor, have yet to be used in medicine. Low lysine levels have been found in patients with Parkinson's, hypothyroidism, kidney disease, asthma and depression. The exact significance of these levels is unclear, yet lysine therapy can normalize the level and has been associated with improvement of some patients with these conditions. Abnormally elevated hydroxylysines have been found in virtually all chronic degenerative diseases and those treated with coumadin therapy. The levels of this stress marker may be improved by high doses of vitamin C. Lysine is particularly useful in therapy for marasmus (wasting) (http://www.dcnutrition.com). Lysine has also been shown to play a role in anaemia, as lysine is suspected to have an effect on the uptake of iron and, subsequently, the concentration of ferritin in blood plasma.
Structure
Data?1601268944
Synonyms
ValueSource
(S)-2,6-Diaminohexanoic acidChEBI
(S)-alpha,epsilon-Diaminocaproic acidChEBI
(S)-LysineChEBI
6-Ammonio-L-norleucineChEBI
KChEBI
L-2,6-Diaminocaproic acidChEBI
L-LysinChEBI
LysChEBI
LysinaChEBI
LysineChEBI
Lysine acidChEBI
LysinumChEBI
2,6-Diaminohexanoic acidKegg
(S)-2,6-DiaminohexanoateGenerator
(S)-a,epsilon-DiaminocaproateGenerator
(S)-a,epsilon-Diaminocaproic acidGenerator
(S)-alpha,epsilon-DiaminocaproateGenerator
(S)-Α,epsilon-diaminocaproateGenerator
(S)-Α,epsilon-diaminocaproic acidGenerator
L-2,6-DiaminocaproateGenerator
2,6-DiaminohexanoateGenerator
(+)-S-LysineHMDB
(S)-2,6-Diamino-hexanoateHMDB
(S)-2,6-Diamino-hexanoic acidHMDB
(S)-a,e-DiaminocaproateHMDB
(S)-a,e-Diaminocaproic acidHMDB
6-Amino-aminutrinHMDB
6-Amino-L-norleucineHMDB
a-LysineHMDB
alpha-LysineHMDB
AminutrinHMDB
H-Lys-OHHMDB
L-(+)-LysineHMDB
L-2,6-DiainohexanoateHMDB
L-2,6-Diainohexanoic acidHMDB
L-LysHMDB
Acetate, lysineHMDB
EnisylHMDB
Lysine hydrochlorideHMDB
L LysineHMDB
Lysine acetateHMDB
Chemical FormulaC6H14N2O2
Average Molecular Weight146.1876
Monoisotopic Molecular Weight146.105527702
IUPAC Name(2S)-2,6-diaminohexanoic acid
Traditional NameL-lysine
CAS Registry Number56-87-1
SMILES
NCCCC[C@H](N)C(O)=O
InChI Identifier
InChI=1S/C6H14N2O2/c7-4-2-1-3-5(8)6(9)10/h5H,1-4,7-8H2,(H,9,10)/t5-/m0/s1
InChI KeyKDXKERNSBIXSRK-YFKPBYRVSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as l-alpha-amino acids. These are alpha amino acids which have the L-configuration of the alpha-carbon atom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentL-alpha-amino acids
Alternative Parents
Substituents
  • L-alpha-amino acid
  • Medium-chain fatty acid
  • Amino fatty acid
  • Fatty acid
  • Fatty acyl
  • Amino acid
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Organic oxide
  • Organopnictogen compound
  • Organic oxygen compound
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Primary aliphatic amine
  • Carbonyl group
  • Organic nitrogen compound
  • Amine
  • Hydrocarbon derivative
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect

Adverse health effect

Disposition

Biological location

Source

Route of exposure

Process

Naturally occurring process

Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point224.5 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility1000 mg/mLNot Available
LogP-3.05HANSCH,C ET AL. (1995)
Experimental Spectral Properties

Experimental Collision Cross Sections

PredictorAdduct TypeData SourceCCS Value (Å2)Reference
DeepCCS[M-H]-Astarita_neg132.730932474
DeepCCS[M+H]+Astarita_pos127.730932474
DeepCCS[M+H]+Baker131.830932474
DeepCCS[M+H]+McLean131.73830932474
DeepCCS[M+H]+MetCCS_test_pos130.73530932474
AllCCS[M-H]-Not Available134.2http://allccs.zhulab.cn/database/detail?ID=AllCCS00000507
AllCCS[M+H]+Not Available131.3http://allccs.zhulab.cn/database/detail?ID=AllCCS00000507
Predicted Molecular Properties
PropertyValueSource
Water Solubility105 g/LALOGPS
logP10(-3.8) g/LALOGPS
logP10(-3.2) g/LChemAxon
logS10(-0.14) g/LALOGPS
pKa (Strongest Acidic)2.74ChemAxon
pKa (Strongest Basic)10.29ChemAxon
Physiological Charge1ChemAxon
Hydrogen Acceptor Count4ChemAxon
Hydrogen Donor Count3ChemAxon
Polar Surface Area89.34 ŲChemAxon
Rotatable Bond Count5ChemAxon
Refractivity37.81 m³·mol⁻¹ChemAxon
Polarizability15.84 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Spectral Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference
DarkChem[M+H]+133.39431661259
DarkChem[M+H]+133.39431661259
DarkChem[M+H]+133.39431661259
DarkChem[M-H]-130.28831661259
DarkChem[M-H]-130.28831661259
DarkChem[M-H]-130.28831661259
AllCCS[M+H]+133.232859911
AllCCS[M-H]-128.97132859911

Predicted Kovats Retention Indices

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
L-Lysine,1TMS,#1C[Si](C)(C)OC(=O)[C@@H](N)CCCCN1465.0Semi standard non polarhttps://arxiv.org/abs/1905.12712
L-Lysine,1TMS,#2C[Si](C)(C)NCCCC[C@H](N)C(=O)O1618.2Semi standard non polarhttps://arxiv.org/abs/1905.12712
L-Lysine,1TMS,#3C[Si](C)(C)N[C@@H](CCCCN)C(=O)O1589.5Semi standard non polarhttps://arxiv.org/abs/1905.12712
L-Lysine,1TBDMS,#1CC(C)(C)[Si](C)(C)OC(=O)[C@@H](N)CCCCN1694.5Semi standard non polarhttps://arxiv.org/abs/1905.12712
L-Lysine,1TBDMS,#2CC(C)(C)[Si](C)(C)NCCCC[C@H](N)C(=O)O1876.9Semi standard non polarhttps://arxiv.org/abs/1905.12712
L-Lysine,1TBDMS,#3CC(C)(C)[Si](C)(C)N[C@@H](CCCCN)C(=O)O1836.8Semi standard non polarhttps://arxiv.org/abs/1905.12712
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental GC-MSGC-MS Spectrum - L-Lysine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)splash10-00di-3910000000-98c565675de67aa879002014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Lysine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-0ab9-1910000000-87ef8534f592041f50f22014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Lysine GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (4 TMS)splash10-0a4i-1921000000-84f7815b0f650fa174442014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Lysine GC-MS (3 TMS)splash10-001i-9600000000-823408dba509cb204acf2014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Lysine GC-MS (3 TMS)splash10-00di-3910000000-4f5578af5e7d8b6c49f72014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Lysine GC-MS (4 TMS)splash10-0adi-1921000000-4e56d95e623e792f9e6b2014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Lysine EI-B (Non-derivatized)splash10-00di-0921000000-eeb49e57bc1a751930582017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Lysine EI-B (Non-derivatized)splash10-0ab9-0921000000-ebb902be0f3754225b2f2017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Lysine GC-EI-TOF (Non-derivatized)splash10-00di-3910000000-98c565675de67aa879002017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Lysine GC-EI-TOF (Non-derivatized)splash10-0ab9-1910000000-87ef8534f592041f50f22017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Lysine GC-EI-QQ (Non-derivatized)splash10-0fdk-3923000000-15b84c2649c1b0455de12017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Lysine GC-EI-TOF (Non-derivatized)splash10-0a4i-1921000000-84f7815b0f650fa174442017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Lysine GC-MS (Non-derivatized)splash10-001i-9600000000-823408dba509cb204acf2017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Lysine GC-MS (Non-derivatized)splash10-00di-3910000000-4f5578af5e7d8b6c49f72017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Lysine GC-MS (Non-derivatized)splash10-0adi-1921000000-4e56d95e623e792f9e6b2017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Lysine GC-EI-TOF (Non-derivatized)splash10-0abi-1900000000-9ad174122e4d6e003eb82017-09-12HMDB team, MONA, MassBankView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - L-Lysine GC-MS (Non-derivatized) - 70eV, Positivesplash10-0089-9100000000-974cc55c9130ed5213eb2016-09-22Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - L-Lysine GC-MS (1 TMS) - 70eV, Positivesplash10-0ue9-9700000000-57b24ae819b6ec26bfd22017-10-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - L-Lysine GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Lysine LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negative-QTOFsplash10-0002-0900000000-a997e8098743579088802012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Lysine LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negative-QTOFsplash10-0002-2900000000-f64110414f82c93e1fe82012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Lysine LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negative-QTOFsplash10-014m-9200000000-33a38e6370811c5ddc822012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Lysine LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negative-QTOFsplash10-0006-9000000000-3a8b0b6e62f5c66d37202012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Lysine LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negative-QTOFsplash10-0006-9000000000-d5167570d11d77fd541e2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Lysine LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negative-QTOFsplash10-0002-0900000000-b4825cc64fcb830c69672012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Lysine LC-ESI-QQ , negative-QTOFsplash10-0002-0900000000-a997e8098743579088802017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Lysine LC-ESI-QQ , negative-QTOFsplash10-0002-2900000000-c4ff775e954fa48b2c192017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Lysine LC-ESI-QQ , negative-QTOFsplash10-014m-9200000000-33a38e6370811c5ddc822017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Lysine LC-ESI-QQ , negative-QTOFsplash10-0006-9000000000-3a8b0b6e62f5c66d37202017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Lysine LC-ESI-QQ , negative-QTOFsplash10-0006-9000000000-d5167570d11d77fd541e2017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Lysine LC-ESI-QTOF , negative-QTOFsplash10-0002-0900000000-b4825cc64fcb830c69672017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Lysine 20V, Negative-QTOFsplash10-0a4i-9000000000-0f5102647fc779107d702021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Lysine 40V, Negative-QTOFsplash10-06r6-9000000000-5d8c0c807f68164990592021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Lysine 35V, Negative-QTOFsplash10-0002-0900000000-3ecbec789346137a82532021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Lysine 20V, Negative-QTOFsplash10-0002-9000000000-4c4ede391d257c24406c2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Lysine 10V, Negative-QTOFsplash10-0a4i-9400000000-cda5107394204cc5ffa52021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Lysine 33V, Negative-QTOFsplash10-014j-9300000000-02b0e010b98a463348b42021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Lysine 33V, Negative-QTOFsplash10-014j-9700000000-9e4d99d347ca404a2fec2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Lysine 35V, Negative-QTOFsplash10-0002-0900000000-da001b514f81735845bd2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Lysine 35V, Negative-QTOFsplash10-0002-0900000000-487a4327777e2a6fa94d2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Lysine Quattro_QQQ 10V, Positive-QTOF (Annotated)splash10-003r-8900000000-470a0beb4f338ed89bca2012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Lysine Quattro_QQQ 25V, Positive-QTOF (Annotated)splash10-001i-9000000000-74e9193d9d33c2509bfa2012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Lysine Quattro_QQQ 40V, Positive-QTOF (Annotated)splash10-0a59-9000000000-822c4e78250fffa56e392012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Lysine LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOFsplash10-0002-0900000000-04f9a62a77fb5a37ca222012-08-31HMDB team, MONAView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Predicted 1D NMR1H NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Experimental 1D NMR1H NMR Spectrum (1D, 500 MHz, H2O, experimental)2021-10-10Wishart LabView Spectrum
Experimental 1D NMR13C NMR Spectrum (1D, 400 MHz, H2O, experimental)2021-10-10Wishart LabView Spectrum
Experimental 2D NMR[1H, 1H]-TOCSY 2D NMR Spectrum (experimental)2012-12-04Wishart LabView Spectrum
Experimental 2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, experimental)2012-12-05Wishart LabView Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Mitochondria
  • Nucleus
  • Peroxisome
Biospecimen Locations
  • Blood
  • Breast Milk
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Sweat
  • Urine
Tissue Locations
  • All Tissues
  • Placenta
  • Prostate
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified190.0 +/- 60.0 uMNewborn (0-30 days old)Not SpecifiedNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified164.0 +/- 28.0 uMChildren (1-13 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified198.0 +/- 31.0 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified183.0 +/- 34.0 uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified71-151 uMAdult (>18 years old)Not SpecifiedNormal
    • Lysinuric Protein...
details
BloodDetected and Quantified113-269 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
BloodDetected and Quantified441 (384-516) uMNewborn (0-30 days old)Not Available
Normal
details
BloodDetected and Quantified120-203 uMNewborn (0-30 days old)Not SpecifiedNormal details
BloodDetected and Quantified112-231 uMChildren (1-13 years old)BothNormal details
BloodDetected and Quantified85-241 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified189 +/- 27 uMAdult (>18 years old)MaleNormal details
BloodDetected and Quantified238 (192-295) uMInfant (0-1 year old)Not Available
Normal
details
BloodDetected and Quantified161 +/- 21 uMAdult (>18 years old)FemaleNormal details
BloodDetected and Quantified105.100 +/- 30.100 uMNot SpecifiedNot SpecifiedNormal details
BloodDetected and Quantified434.0 +/- 23.0 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified110.0-240.0 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified197.4 +/- 30.7 uMAdult (>18 years old)BothNormal details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified197 (184-216) uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified56-256 uMChildren (1 - 13 years old)BothNormal details
BloodDetected and Quantified82 - 224 uMChildren (1 - 13 years old)Not SpecifiedNormal details
BloodDetected and Quantified92-325 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified40-163 uMNot SpecifiedNot SpecifiedNormal details
BloodDetected and Quantified127.8(108.8-147.7) uMChildren (1-13 uears old)Both
Normal
details
BloodDetected and Quantified71-272 uMNewborn (0-30 days old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified37-228 uMInfant (1 - 3 months old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified85-250 uMChildren (3 months - 6 years old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified108-270 uMChildren (6 - 18 years old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified178.6 +/- 58.2 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified188.0 (156.0-220.0) uMAdult (>18 years old)BothNormal details
Breast MilkDetected and Quantified11.1 +/- 8.8 uMAdult (>18 years old)Female
Normal
details
Cerebrospinal Fluid (CSF)Detected and Quantified21.7 +/- 3.7 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
Cerebrospinal Fluid (CSF)Detected and Quantified29.0 +/- 13.0 uMNot SpecifiedBothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified10-28 uMChildren (1-13 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified14.100-25.300 uMNot SpecifiedNot SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified6-37 uMChildren (0 - 10 years old)Both
Normal
    • BC Children's Hos...
details
Cerebrospinal Fluid (CSF)Detected and Quantified27.7 +/- 8.0 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified12-43 uMAdolescent (>11 years old)Both
Normal
    • BC Children's Hos...
details
Cerebrospinal Fluid (CSF)Detected and Quantified31.5 +/- 5.7 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified25.7 +/- 5.3 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified23.9 (18.1-29.7) uMAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedInfant (0-1 year old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)FemaleNormal details
FecesDetected but not QuantifiedNot QuantifiedChildren (1-13 years old)BothNormal details
FecesDetected and Quantified3078.236 (2052.158-4104.315) nmol/g wet fecesInfant (0-1 year old)Not Specified
Normal
details
FecesDetected and Quantified4697.161 (1983.752-7410.569) nmol/g wet fecesInfant (0-1 year old)Not Specified
Normal
details
FecesDetected and Quantified2964.228 (1664.528-4263.928) nmol/g wet fecesInfant (0-1 year old)Not Specified
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedBoth
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Not Specified
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedNot Specified
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)FemaleNormal details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)FemaleNormal details
FecesDetected and Quantified540 +/- 410 nmol/g wet fecesAdult (>18 years old)Both
Normal
details
FecesDetected and Quantified40 +/- 30 nmol/g wet fecesAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedNot Specified
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
SalivaDetected and Quantified59.20 +/- 46.76 uMAdult (>18 years old)FemaleNormal details
SalivaDetected and Quantified45.1 +/- 45.0 uMAdult (>18 years old)Male
Normal
    • Sugimoto et al. (...
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
SalivaDetected and Quantified19.2 +/- 12.8 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified9.8 +/- 5.2 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified9.2 +/- 4.1 uMAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified2.2 +/- 3.8 uMAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified37.76 +/- 18.10 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
details
SalivaDetected and Quantified3.7 +/- 6.3 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified>10 uMAdult (>18 years old)BothNormal details
SweatDetected and Quantified< 10 uMAdult (60 years old)Male
Normal
details
SweatDetected and Quantified< 10 uMAdult (40 years old)Male
Normal
details
SweatDetected but not QuantifiedNot QuantifiedAdult BothNormal details
UrineDetected and Quantified31.45 +/- 52.32 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
UrineDetected and Quantified10.5-140.5 umol/mmol creatinineNewborn (0-30 days old)BothNormal
    • López Hernández Y...
details
UrineDetected and Quantified38.05 +/- 15.47 umol/mmol creatinineNewborn (0-30 days old)FemaleNormal
    • López Hernández Y...
details
UrineDetected and Quantified51.37 +/- 33.33 umol/mmol creatinineNewborn (0-30 days old)MaleNormal
    • López Hernández Y...
details
UrineDetected and Quantified1.92-31.22 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified23.654 +/- 19.414 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected and Quantified1.13-27.15 umol/mmol creatinineAdolescent (13-18 years old)Not SpecifiedNormal details
UrineDetected and Quantified3.42-75.29 umol/mmol creatinineAdult (>18 years old)BothNormal
    • David F. Putnam C...
details
UrineDetected and Quantified5.2-16 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
UrineDetected and Quantified18.09 - 217.98 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified8.71 - 199.10 umol/mmol creatinineInfant (1 - 6 months old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified5.99 - 94.63 umol/mmol creatinineInfant (6 months - <1 year old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified10.06 - 81.18 umol/mmol creatinineChildren (1 - 2 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified1.92 - 50.31 umol/mmol creatinineChildren (2 - 4 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified1.92 - 79.82 umol/mmol creatinineChildren (4 - 13 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 66.37 umol/mmol creatinineAdolescent (13 - 21 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 66.37 umol/mmol creatinineAdult (>21 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified1.70-30.65 umol/mmol creatinineAdult (>18 years old)Not SpecifiedNormal details
UrineDetected and Quantified10.9(2.105-19.7) umol/mmol creatinineAdult (>18 years old)BothNormal
      Not Available
details
UrineDetected and Quantified29.867 +/- 22.0667 umol/mmol creatinineAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified7.467 +/- 6.360 umol/mmol creatinineChildren (1 - 13 years old)FemaleNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified17.2 (3.7-51.3) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified1.32-26.75 umol/mmol creatinineNewborn (0-30 days old)Male
Normal
details
UrineDetected and Quantified32.800 +/- 27.667 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified7.70-30.13 umol/mmol creatinineNewborn (0-30 days old)Female
Normal
details
UrineDetected and Quantified10.87 +/- 8.80 umol/mmol creatinineNewborn (0-30 days old)Male
Normal
details
UrineDetected and Quantified13.09 +/- 5.90 umol/mmol creatinineChildren (0-2 years old)Male
Normal
details
UrineDetected and Quantified13.44 +/- 5.50 umol/mmol creatinineChildren (0-2 years old)Male
Normal
details
UrineDetected and Quantified16.06 +/- 7.48 umol/mmol creatinineNewborn (0-30 days old)Female
Normal
details
UrineDetected and Quantified2.15-224.84 umol/mmol creatinineInfant (0-1 year old)Not SpecifiedNormal details
UrineDetected and Quantified9.0667 +/- 5.707 umol/mmol creatinineChildren (1 - 13 years old)MaleNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified10-68 umol/mmol creatinineChildren (1 - 13 years old)BothNormal details
UrineDetected and Quantified19.335-100.541 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified11.601 +/- 3.867 umol/mmol creatinineAdult (>18 years old)Not SpecifiedNormal details
UrineDetected and Quantified11.601 +/- 3.867 umol/mmol creatinineNot SpecifiedNot SpecifiedNormal details
UrineDetected and Quantified3.1 +/- 1.5 umol/mmol creatinineInfant (1 - 6 months old)Both
Normal
details
UrineDetected and Quantified3.2 +/- 1.9 umol/mmol creatinineInfant (6 months - <1 year old)Both
Normal
details
UrineDetected and Quantified4.4 +- 1.6 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified24.647-202.943 umol/mmol creatinineInfant (0-1 year old)Not SpecifiedNormal details
UrineDetected and Quantified1.933 +/- 1.0741 umol/mmol creatinineNot SpecifiedNot SpecifiedNormal details
UrineDetected and Quantified10.28 +/- 5.34 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified68.22 (12-124.44) umol/mmol creatinineNewborn (0-30 days old)BothNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified32.0 +/- 27.0 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified29.0 +/- 21.7 umol/mmol creatinineAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified17.9 (3.6-56.1) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified2.83-84.047 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified5.6-15 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified7-58 umol/mmol creatinineNot SpecifiedNot SpecifiedNormal details
UrineDetected and Quantified3-170 umol/mmol creatinineInfant (0-1 year old)Both
Normal
details
UrineDetected and Quantified3-170 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified4.7 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified34.8063 +/- 26.0145 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected and Quantified1.58-34.73 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified226.12 +/- 158.28 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified407.02 +/- 260.04 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified520.08 +/- 327.87 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified0-69 umol/mmol creatinineChildren (1-13 years old)BothNormal details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Oesophageal cancer
details
BloodDetected and Quantified128.2 +/- 55.3 uMAdult (>18 years old)BothHeart Transplant details
BloodDetected and Quantified70 (32-179) uMAdult (>18 years old)Not SpecifiedLysinuric protein intolerance
    • Lysinuric Protein...
details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal cancer details
BloodDetected and Quantified180.5 (142-326) uMChildren (1-13 years old)MaleLipoyltransferase 1 Deficiency details
BloodDetected and Quantified220-384 uMNewborn (0-30 days old)BothCitrullinemia type II, neonatal-onset details
BloodDetected and Quantified1208-1472 uMChildren (1-13 years old)BothHyperlysinemia I, familial details
BloodDetected and Quantified713 uMInfant (0-1 year old)Not AvailablePyruvate carboxylase deficiency details
BloodDetected and Quantified136 uMChildren (1-13 years old)Not AvailablePyruvate carboxylase deficiency details
BloodDetected and Quantified135-831 uMInfant (0-1 year old)Male2,4-dienoyl-CoA reductase deficiency details
BloodDetected and Quantified206 +/- 29 uMAdult (>18 years old)MaleSchizophrenia details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
BloodDetected and Quantified186 +/- 28 uMAdult (>18 years old)FemaleSchizophrenia details
BloodDetected and Quantified103.04 (27.01) uMAdult (>18 years old)FemalePregnancy with fetus having congenital heart defect details
BloodDetected and Quantified97.44 (29.48) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified82.9 uMChildren (1-13 years old)Male
Alpha-aminoadipic and alpha-ketoadipic aciduria
details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Pancreatic cancer
details
BloodDetected and Quantified147.5(112.8-185.0) uMChildren (1-13 uears old)Both
Environmental enteric dysfunction
details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Pancreatic ductal adenocarcinoma
details
BloodDetected and Quantified107 uMInfant (0-1 year old)MaleFumaric aciduria details
BloodDetected and Quantified116 (89.4-136) uMAdult (>18 years old)Both
Lysinuric protein intolerance
details
BloodDetected and Quantified147 +/- 42 uMChildren (1 - 13 years old)MaleHistidinuria details
BloodDetected and Quantified185-396 uMChildren (1-13 years old)BothL-2-hydroxyglutaric aciduria details
BloodDetected and Quantified216.7 +/- 54.5 uMElderly (>65 years old)BothAlzheimer's disease details
BloodDetected and Quantified24-73 uMChildren (1 - 13 years old)BothLysinuric protein intolerance details
BloodDetected and Quantified128.0 +/- 9.0 uMAdult (>18 years old)BothRefractory localization-related epilepsy (RLE) details
Cerebrospinal Fluid (CSF)Detected and Quantified226 uMInfant (0-1 year old)Male2,4-dienoyl-CoA reductase deficiency details
Cerebrospinal Fluid (CSF)Detected and Quantified44.6 +/- 10.8 uMAdult (>18 years old)BothAlzheimer's disease details
Cerebrospinal Fluid (CSF)Detected and Quantified60.000-94.500 uMChildren (1-13 years old)Female
L-2-hydroxyglutaric aciduria
details
Cerebrospinal Fluid (CSF)Detected and Quantified26.9 +/- 11.1 uMChildren (1-13 years old)Not SpecifiedLeukemia details
Cerebrospinal Fluid (CSF)Detected and Quantified21.9 +/- 6.2 uMChildren (1-13 years old)Not Specified
Leukemia
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal Cancer details
FecesDetected but not QuantifiedNot QuantifiedChildren (1-13 years old)BothAutism details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Irritable bowel syndrome
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Ulcerative colitis
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)FemaleMyalgic encephalomyelitis/chronic fatigue syndrome details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedCrohns disease details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedUlcerative colitis details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedUnclassified IBD details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal cancer details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothIrritable Bowel Syndrome details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Crohn’s Disease
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Ulcerative Colitis
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothCrohn's disease details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothUlcerative colitis details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal cancer details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MaleAttachment loss  details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MaleMissing teeth details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MalePeriodontal Probing Depth details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MaleSupragingival Calculus details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothOral cancer details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)FemaleBreast cancer details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Not SpecifiedPancreatic cancer details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Not SpecifiedPeriodontal diseases details
SalivaDetected and Quantified45.55 +/- 15.63 uMAdult (>18 years old)MaleAlzheimer's disease details
SalivaDetected and Quantified41.71 +/- 22.55 uMAdult (>18 years old)MaleFrontotemporal lobe dementia details
SalivaDetected and Quantified37.38 +/- 19.11 uMAdult (>18 years old)BothLewy body disease details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal adenoma
details
UrineDetected and Quantified1.0489-5.518 umol/mmol creatinineInfant (0-1 year old)FemaleCarbamoyl Phosphate Synthetase Deficiency details
UrineDetected and Quantified34.402 +/- 30.429 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
UrineDetected and Quantified130.200-1873.600 umol/mmol creatinineAdult (>18 years old)Both
Cystinuria
details
UrineDetected and Quantified18.0 (7.0-29.0) umol/mmol creatinineAdult (>18 years old)BothCarbamoyl Phosphate Synthetase Deficiency
    • MetaGene: Metabol...
details
UrineDetected and Quantified2750.0 (500.0-5000.0) umol/mmol creatinineChildren (1-13 years old)BothCarbamoyl phosphate synthetase deficiency
    • MetaGene: Metabol...
details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Bladder cancer
details
UrineDetected and Quantified0.0039 - 0.0116 umol/mmol creatinineAdult (>18 years old)BothADPKD details
UrineDetected and Quantified1779 umol/mmol creatinineInfant (0-1 year old)Male2,4-dienoyl-CoA reductase deficiency details
UrineDetected and Quantified2050-18736 umol/mmol creatinineAdult (>18 years old)BothHyperlysinuria details
UrineDetected and Quantified1413.757 umol/mmol creatinineChildren (1-13 years old)FemaleHyperlysinuria details
UrineDetected and Quantified41.840-220.880 umol/mmol creatinineChildren (1-13 years old)FemaleDibasic Amino Aciduria I details
UrineDetected and Quantified30.704-356.533 umol/mmol creatinineAdult (>18 years old)BothDibasic Amino Aciduria I details
UrineDetected and Quantified3.414 umol/mmol creatinineChildren (1-13 years old)Male
Alpha-aminoadipic and alpha-ketoadipic aciduria
details
UrineDetected and Quantified111.677-220.880 umol/mmol creatinineChildren (1 - 13 years old)FemaleHyperdibasic aminoaciduria I details
UrineDetected and Quantified1272.2 umol/mmol creatinineAdult (>18 years old)Both
Cystinuria
details
UrineDetected and Quantified268.300-365.200 umol/mmol creatinineInfant (0-1 year old)Not SpecifiedFumaric aciduria details
UrineDetected and Quantified30.704-137.431 umol/mmol creatinineAdult (>18 years old)BothHyperdibasic aminoaciduria I details
UrineDetected and Quantified548-1317.200 umol/mmol creatinineChildren (1 - 13 years old)BothLysinuric protein intolerance details
UrineDetected and Quantified5.5 +/- 1.74 umol/mmol creatinineAdult (>18 years old)BothAlzheimer's disease details
UrineDetected and Quantified36-168 umol/mmol creatinineChildren (1-13 years old)BothL-2-hydroxyglutaric aciduria details
UrineDetected and Quantified28.0899 +/- 25.2794 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
UrineDetected and Quantified18.5215 +/- 11.0832 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Gastroesophageal reflux disease
    • Mordechai, Hien, ...
details
UrineDetected and Quantified101 +/- 13 umol/mmol creatinineAdult (>18 years old)BothPropionic acidemia details
UrineDetected and Quantified452.1 +/- 60.2 umol/mmol creatinineAdult (>18 years old)BothTyrosinemia I details
UrineDetected and Quantified321.8 +/- 15.4 umol/mmol creatinineAdult (>18 years old)BothTyrosinemia I details
UrineDetected and Quantified397.5 +/- 16.6 umol/mmol creatinineAdult (>18 years old)BothAminoaciduria details
UrineDetected and Quantified1384-1742 umol/mmol creatinineChildren (1-13 years old)BothHyperlysinemia I, familial details
Associated Disorders and Diseases
Disease References
Refractory localization-related epilepsy
  1. 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 ]
Alzheimer's disease
  1. Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed:17031479 ]
  2. 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 ]
Schizophrenia
  1. 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 ]
Pregnancy
  1. 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 ]
Lysinuric protein intolerance
  1. Kurko J, Tringham M, Tanner L, Nanto-Salonen K, Vaha-Makila M, Nygren H, Poho P, Lietzen N, Mattila I, Olkku A, Hyotylainen T, Oresic M, Simell O, Niinikoski H, Mykkanen J: Imbalance of plasma amino acids, metabolites and lipids in patients with lysinuric protein intolerance (LPI). Metabolism. 2016 Sep;65(9):1361-75. doi: 10.1016/j.metabol.2016.05.012. Epub 2016 May 27. [PubMed:27506743 ]
  2. Habib A, Azize NA, Yakob Y, Md Yunus Z, Wee TK: Biochemical and molecular characteristics of Malaysian patients with lysinuric protein intolerance. Malays J Pathol. 2016 Dec;38(3):305-310. [PubMed:28028301 ]
  3. Olli Simell (1995). Lysinuric Protein Intolerance and Other Cationic Aminoacidurias. The metabolic and molecular bases of inherited disease, 7/e; Editors: C.R.Scriver, A.L.Beaudet, W.S.Sly, D.Valle; McGraw-Hill Inc. (1995) DOI: 10.1036/ommbid.225. McGraw-Hill Inc..
Fumarase deficiency
  1. Allegri G, Fernandes MJ, Scalco FB, Correia P, Simoni RE, Llerena JC Jr, de Oliveira ML: Fumaric aciduria: an overview and the first Brazilian case report. J Inherit Metab Dis. 2010 Aug;33(4):411-9. doi: 10.1007/s10545-010-9134-2. Epub 2010 Jun 15. [PubMed:20549362 ]
Histidinemia
  1. Nyhan WL, Hilton S: Histidinuria: defective transport of histidine. Am J Med Genet. 1992 Nov 15;44(5):558-61. [PubMed:1481808 ]
Citrullinemia type II, neonatal-onset
  1. Ohura T, Kobayashi K, Tazawa Y, Nishi I, Abukawa D, Sakamoto O, Iinuma K, Saheki T: Neonatal presentation of adult-onset type II citrullinemia. Hum Genet. 2001 Feb;108(2):87-90. [PubMed:11281457 ]
Pyruvate carboxylase deficiency
  1. 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 ]
Lipoyltransferase 1 Deficiency
  1. 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 ]
Leukemia
  1. 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 ]
L-2-Hydroxyglutaric aciduria
  1. Barbot C, Fineza I, Diogo L, Maia M, Melo J, Guimaraes A, Pires MM, Cardoso ML, Vilarinho L: L-2-Hydroxyglutaric aciduria: clinical, biochemical and magnetic resonance imaging in six Portuguese pediatric patients. Brain Dev. 1997 Jun;19(4):268-73. [PubMed:9187477 ]
Crohn's disease
  1. 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 ]
  2. 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 ]
Ulcerative colitis
  1. 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 ]
  2. 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 ]
  3. 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 ]
Irritable bowel syndrome
  1. 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 ]
  2. 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 ]
Colorectal cancer
  1. 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 ]
  2. 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 ]
  3. 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 ]
  4. 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 ]
  5. 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 ]
  6. 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 ]
Autism
  1. 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 ]
Perillyl alcohol administration for cancer treatment
  1. 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
  1. 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 ]
  2. 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
  1. 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 ]
Frontotemporal dementia
  1. 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
  1. 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 ]
Attachment loss
  1. Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Missing teeth
  1. Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Periodontal Probing Depth
  1. Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Supragingival Calculus
  1. Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Carbamoyl Phosphate Synthetase Deficiency
  1. Freeman JM, Nicholson JF, Schimke RT, Rowland LP, Carter S: Congenital hyperammonemia. Association with hyperglycinemia and decreased levels of carbamyl phosphate synthetase. Arch Neurol. 1970 Nov;23(5):430-7. [PubMed:5471650 ]
  2. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Autosomal dominant polycystic kidney disease
  1. Gronwald W, Klein MS, Zeltner R, Schulze BD, Reinhold SW, Deutschmann M, Immervoll AK, Boger CA, Banas B, Eckardt KU, Oefner PJ: Detection of autosomal dominant polycystic kidney disease by NMR spectroscopic fingerprinting of urine. Kidney Int. 2011 Jun;79(11):1244-53. doi: 10.1038/ki.2011.30. Epub 2011 Mar 9. [PubMed:21389975 ]
Propionic acidemia
  1. Gronwald W, Klein MS, Kaspar H, Fagerer SR, Nurnberger N, Dettmer K, Bertsch T, Oefner PJ: Urinary metabolite quantification employing 2D NMR spectroscopy. Anal Chem. 2008 Dec 1;80(23):9288-97. doi: 10.1021/ac801627c. [PubMed:19551947 ]
Tyrosinemia I
  1. Gronwald W, Klein MS, Kaspar H, Fagerer SR, Nurnberger N, Dettmer K, Bertsch T, Oefner PJ: Urinary metabolite quantification employing 2D NMR spectroscopy. Anal Chem. 2008 Dec 1;80(23):9288-97. doi: 10.1021/ac801627c. [PubMed:19551947 ]
Eosinophilic esophagitis
  1. (). Mordechai, Hien, and David S. Wishart. .
Hyperdibasic aminoaciduria I
  1. Whelan DT, Scriver CR: Hyperdibasicaminoaciduria: an inherited disorder of amino acid transport. Pediatr Res. 1968 Nov;2(6):525-34. [PubMed:5727921 ]
Cystinuria
  1. Fjellstedt E, Harnevik L, Jeppsson JO, Tiselius HG, Soderkvist P, Denneberg T: Urinary excretion of total cystine and the dibasic amino acids arginine, lysine and ornithine in relation to genetic findings in patients with cystinuria treated with sulfhydryl compounds. Urol Res. 2003 Dec;31(6):417-25. Epub 2003 Oct 25. [PubMed:14586528 ]
Alpha-aminoadipic and alpha-ketoadipic aciduria
  1. Takechi T, Okada T, Wakiguchi H, Morita H, Kurashige T, Sugahara K, Kodama H: Identification of N-acetyl-alpha-aminoadipic acid in the urine of a patient with alpha-aminoadipic and alpha-ketoadipic aciduria. J Inherit Metab Dis. 1993;16(1):119-26. [PubMed:8487492 ]
2,4-dienoyl-CoA reductase deficiency
  1. Houten SM, Denis S, Te Brinke H, Jongejan A, van Kampen AH, Bradley EJ, Baas F, Hennekam RC, Millington DS, Young SP, Frazier DM, Gucsavas-Calikoglu M, Wanders RJ: Mitochondrial NADP(H) deficiency due to a mutation in NADK2 causes dienoyl-CoA reductase deficiency with hyperlysinemia. Hum Mol Genet. 2014 Sep 15;23(18):5009-16. doi: 10.1093/hmg/ddu218. Epub 2014 May 8. [PubMed:24847004 ]
Hyperlysinuria
  1. Omura K, Yamanaka N, Higami S, Matsuoka O, Fujimoto A: Lysine malabsorption syndrome: a new type of transport defect. Pediatrics. 1976 Jan;57(1):102-5. [PubMed:1246485 ]
  2. Fjellstedt E, Harnevik L, Jeppsson JO, Tiselius HG, Soderkvist P, Denneberg T: Urinary excretion of total cystine and the dibasic amino acids arginine, lysine and ornithine in relation to genetic findings in patients with cystinuria treated with sulfhydryl compounds. Urol Res. 2003 Dec;31(6):417-25. Epub 2003 Oct 25. [PubMed:14586528 ]
Hyperlysinemia I, familial
  1. Tondo M, Calpena E, Arriola G, Sanz P, Martorell L, Ormazabal A, Castejon E, Palacin M, Ugarte M, Espinos C, Perez B, Perez-Duenas B, Perez-Cerda C, Artuch R: Clinical, biochemical, molecular and therapeutic aspects of 2 new cases of 2-aminoadipic semialdehyde synthase deficiency. Mol Genet Metab. 2013 Nov;110(3):231-6. doi: 10.1016/j.ymgme.2013.06.021. Epub 2013 Jul 6. [PubMed:23890588 ]
Associated OMIM IDs
DrugBank IDDB00123
Phenol Explorer Compound IDNot Available
FooDB IDFDB000474
KNApSAcK IDC00001378
Chemspider ID5747
KEGG Compound IDC00047
BioCyc IDLYS
BiGG ID33655
Wikipedia LinkLysine
METLIN ID5200
PubChem Compound5962
PDB IDLYS
ChEBI ID18019
Food Biomarker OntologyNot Available
VMH IDLYS_L
MarkerDB IDMDB00000087
Good Scents IDNot Available
References
Synthesis ReferenceRothstein, Morton. DL-Lysine-6-C14 and DL-a-aminoadipic acid-6-C14. Biochemical Preparations (1961), 8 85-8.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. 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 ]
  2. 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 ]
  3. 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 ]
  4. Engelborghs S, Marescau B, De Deyn PP: Amino acids and biogenic amines in cerebrospinal fluid of patients with Parkinson's disease. Neurochem Res. 2003 Aug;28(8):1145-50. [PubMed:12834252 ]
  5. 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 ]
  6. 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 ]
  7. 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 ]
  8. 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 ]
  9. Kranz BR: Detection of rare malignant cells and their apoptotic fragments in cerebrospinal fluid. Lancet. 2000 Oct 7;356(9237):1242-4. [PubMed:11072949 ]
  10. Hajishengallis G, Koga T, Russell MW: Affinity and specificity of the interactions between Streptococcus mutans antigen I/II and salivary components. J Dent Res. 1994 Sep;73(9):1493-502. [PubMed:7523469 ]
  11. Pahler A, Parker J, Dekant W: Dose-dependent protein adduct formation in kidney, liver, and blood of rats and in human blood after perchloroethene inhalation. Toxicol Sci. 1999 Mar;48(1):5-13. [PubMed:10330678 ]
  12. Faraasen S, Voros J, Csucs G, Textor M, Merkle HP, Walter E: Ligand-specific targeting of microspheres to phagocytes by surface modification with poly(L-lysine)-grafted poly(ethylene glycol) conjugate. Pharm Res. 2003 Feb;20(2):237-46. [PubMed:12636162 ]
  13. Brunk E, Sahoo S, Zielinski DC, Altunkaya A, Drager A, Mih N, Gatto F, Nilsson A, Preciat Gonzalez GA, Aurich MK, Prlic A, Sastry A, Danielsdottir AD, Heinken A, Noronha A, Rose PW, Burley SK, Fleming RMT, Nielsen J, Thiele I, Palsson BO: Recon3D enables a three-dimensional view of gene variation in human metabolism. Nat Biotechnol. 2018 Mar;36(3):272-281. doi: 10.1038/nbt.4072. Epub 2018 Feb 19. [PubMed:29457794 ]
  14. 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 25 proteins in total.

Enzymes

General function:
Involved in DNA binding
Specific function:
Histone methyltransferase. Methylates 'Lys-79' of histone H3. Nucleosomes are preferred as substrate compared to free histones. Binds to DNA.
Gene Name:
DOT1L
Uniprot ID:
Q8TEK3
Molecular weight:
164854.41
General function:
Involved in histone-lysine N-methyltransferase activity
Specific function:
Histone methyltransferase that specifically monomethylates 'Lys-4' of histone H3. H3 'Lys-4' methylation represents a specific tag for epigenetic transcriptional activation. Plays a central role in the transcriptional activation of genes such as collagenase or insulin. Recruited by IPF1/PDX-1 to the insulin promoter, leading to activate transcription. Has also methyltransferase activity toward non-histone proteins such as p53/TP53, TAF10, and possibly TAF7 by recognizing and binding the [KR]-[STA]-K in substrate proteins. Monomethylates 'Lys-189' of TAF10, leading to increase the affinity of TAF10 for RNA polymerase II. Monomethylates 'Lys-372' of p53/TP53, stabilizing p53/TP53 and increasing p53/TP53-mediated transcriptional activation.
Gene Name:
SETD7
Uniprot ID:
Q8WTS6
Molecular weight:
40720.595
General function:
Involved in histone-lysine N-methyltransferase activity
Specific function:
Histone methyltransferase. Preferentially methylates 'Lys-36' of histone H3 and 'Lys-20' of histone H4 (in vitro). Transcriptional intermediary factor capable of both negatively or positively influencing transcription, depending on the cellular context.
Gene Name:
NSD1
Uniprot ID:
Q96L73
Molecular weight:
296649.335
General function:
Involved in DNA binding
Specific function:
Histone methyltransferase that specifically trimethylates 'Lys-9' of histone H3. H3 'Lys-9' trimethylation represents a specific tag for epigenetic transcriptional repression by recruiting HP1 (CBX1, CBX3 and/or CBX5) proteins to methylated histones. Mainly functions in euchromatin regions, thereby playing a central role in the silencing of euchromatic genes. H3 'Lys-9' trimethylation is coordinated with DNA methylation. Probably forms a complex with MBD1 and ATF7IP that represses transcription and couples DNA methylation and histone 'Lys-9' trimethylation. Its activity is dependent on MBD1 and is heritably maintained through DNA replication by being recruited by CAF-1. SETDB1 is targeted to histone H3 by TRIM28/TIF1B, a factor recruited by KRAB zinc-finger proteins.
Gene Name:
SETDB1
Uniprot ID:
Q15047
Molecular weight:
143155.6
General function:
Involved in zinc ion binding
Specific function:
Histone methyltransferase that specifically mono- and dimethylates 'Lys-9' of histone H3 (H3K9me1 and H3K9me2, respectively) in euchromatin. H3K9me represents a specific tag for epigenetic transcriptional repression by recruiting HP1 proteins to methylated histones. Also mediates monomethylation of 'Lys-56' of histone H3 (H3K56me1) in G1 phase, leading to promote interaction between histone H3 and PCNA and regulating DNA replication. Also weakly methylates 'Lys-27' of histone H3 (H3K27me). Also required for DNA methylation, the histone methyltransferase activity is not required for DNA methylation, suggesting that these 2 activities function independently. Probably targeted to histone H3 by different DNA-binding proteins like E2F6, MGA, MAX and/or DP1. May also methylate histone H1. In addition to the histone methyltransferase activity, also methylates non-histone proteins: mediates dimethylation of 'Lys-373' of p53/TP53. Also methylates CDYL, WIZ, ACIN1, DNMT1, HDAC1, ERCC6, KLF12 and itself.
Gene Name:
EHMT2
Uniprot ID:
Q96KQ7
Molecular weight:
132369.205
General function:
Involved in zinc ion binding
Specific function:
Histone methyltransferase that specifically mono- and dimethylates 'Lys-9' of histone H3 (H3K9me1 and H3K9me2, respectively) in euchromatin. H3K9me represents a specific tag for epigenetic transcriptional repression by recruiting HP1 proteins to methylated histones. Also weakly methylates 'Lys-27' of histone H3 (H3K27me). Also required for DNA methylation, the histone methyltransferase activity is not required for DNA methylation, suggesting that these 2 activities function independently. Probably targeted to histone H3 by different DNA-binding proteins like E2F6, MGA, MAX and/or DP1. During G0 phase, it probably contributes to silencing of MYC- and E2F-responsive genes, suggesting a role in G0/G1 transition in cell cycle. In addition to the histone methyltransferase activity, also methylates non-histone proteins: mediates dimethylation of 'Lys-373' of p53/TP53.
Gene Name:
EHMT1
Uniprot ID:
Q9H9B1
Molecular weight:
86702.845
General function:
Involved in oxidoreductase activity
Specific function:
Forms hydroxylysine residues in -Xaa-Lys-Gly- sequences in collagens. These hydroxylysines serve as sites of attachment for carbohydrate units and are essential for the stability of the intermolecular collagen cross-links.
Gene Name:
PLOD1
Uniprot ID:
Q02809
Molecular weight:
83549.55
General function:
Involved in oxidoreductase activity
Specific function:
Forms hydroxylysine residues in -Xaa-Lys-Gly- sequences in collagens. These hydroxylysines serve as sites of attachment for carbohydrate units and are essential for the stability of the intermolecular collagen cross-links.
Gene Name:
PLOD2
Uniprot ID:
O00469
Molecular weight:
84685.07
General function:
Involved in oxidoreductase activity
Specific function:
Forms hydroxylysine residues in -Xaa-Lys-Gly- sequences in collagens. These hydroxylysines serve as sites of attachment for carbohydrate units and are essential for the stability of the intermolecular collagen cross-links.
Gene Name:
PLOD3
Uniprot ID:
O60568
Molecular weight:
84784.505
General function:
Involved in hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds, in linear amides
Specific function:
Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation.
Gene Name:
BTD
Uniprot ID:
P43251
Molecular weight:
61132.43
Reactions
Biocytin + Water → Biotin + L-Lysinedetails

Only showing the first 10 proteins. There are 25 proteins in total.