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Record Information

Version

5.0

Status

Detected and Quantified

Creation Date

2005-11-16 15:48:42 UTC

Update Date

2023-05-30 20:55:49 UTC

HMDB ID

HMDB0000317

Secondary Accession Numbers

HMDB00317

Metabolite Identification

Common Name

2-Hydroxy-3-methylpentanoic acid

Description

2-Hydroxy-3-methylpentanoic acid or 2-hydroxy-3-methylvaleric acid (HMVA) is an organic acid generated by L-isoleucine metabolism. It is derived from the reduction of 2-Keto-3-methylvaleric acid (KMVA), possibly through the action of a lactate dehydrogenase (PMID: 1429566 ). There are 4 stereoisomers of HMVA (2S,3S-HMVA, 2R,3R-HMVA, 2S,3R-HMVA and 2R,3S-HMVA), of which the 2S,3S and 2S,3R derivatives are generally separable. HMVA is found in the urine and blood of normal individuals but in very elevated levels in patients with maple syrup urine disease (MSUD) (PMID: 1429566 ). Maple syrup urine disease (MSUD) is an inherited metabolic disease predominantly characterized by neurological dysfunction including psychomotor/delay/mental retardation.

Structure

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

View in JSmol View Stereo Labels

Synonyms

Value	Source
2-Hydroxy-3-methylpentanoate	Generator
(2R,3R)-2-Hydroxy-3-methyl-pentanoate	HMDB
(2R,3R)-2-Hydroxy-3-methyl-pentanoic acid	HMDB
(2R,3R)-2-Hydroxy-3-methylpentanoate	HMDB
(2R,3R)-2-Hydroxy-3-methylpentanoic acid	HMDB
2-Hydroxy-3-methyl-pentanoate	HMDB
2-Hydroxy-3-methyl-pentanoic acid	HMDB
2-Hydroxy-3-methyl-valerate	HMDB
2-Hydroxy-3-methyl-valeric acid	HMDB
2-Hydroxy-3-methylvalerate	HMDB
2-Hydroxy-3-methylvaleric acid	HMDB
a-Hydroxy-b-methylvalerate	HMDB
a-Hydroxy-b-methylvaleric acid	HMDB
alpha-Hydroxy-beta-methylvalerate	HMDB
alpha-Hydroxy-beta-methylvaleric acid	HMDB
HMVA	HMDB

Chemical Formula

C₆H₁₂O₃

Average Molecular Weight

132.1577

Monoisotopic Molecular Weight

132.07864425

IUPAC Name

(2R,3R)-2-hydroxy-3-methylpentanoic acid

Traditional Name

2-hydroxy-3-methyl-valerate

CAS Registry Number

488-15-3

SMILES

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

InChI Identifier

InChI=1S/C6H12O3/c1-3-4(2)5(7)6(8)9/h4-5,7H,3H2,1-2H3,(H,8,9)/t4-,5-/m1/s1

InChI Key

RILPIWOPNGRASR-RFZPGFLSSA-N

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as hydroxy fatty acids. These are fatty acids in which the chain bears a hydroxyl group.

Kingdom

Organic compounds

Super Class

Lipids and lipid-like molecules

Class

Fatty Acyls

Sub Class

Fatty acids and conjugates

Direct Parent

Hydroxy fatty acids

Alternative Parents

Substituents

Branched fatty acid
Methyl-branched fatty acid
Hydroxy fatty acid
Alpha-hydroxy acid
Hydroxy acid
Monosaccharide
Secondary alcohol
Monocarboxylic acid or derivatives
Carboxylic acid
Carboxylic acid derivative
Alcohol
Hydrocarbon derivative
Carbonyl group
Organic oxide
Organic oxygen compound
Organooxygen compound
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

Hydroxy fatty acids (LMFA01050380 )

Ontology

Physiological effect

Health effect

Digestive system disorder

Tooth Decay (HMDB: HMDB0000317)

Disposition

Biological location

Non-excretory biofluid

Biofluid or Excreta

Blood (PMID: 6422161)
Saliva (PMID: 22308371)
Cerebrospinal Fluid (CSF) (PMID: 6422161)

Cellular substructure

Organelle

Adiposome (HMDB: HMDB0000317)

Excreta

Biofluid or Excreta

Urine (PMID: 24023812)
Feces (PMID: 27275383)

Route of exposure

Enteral

Ingestion (HMDB: HMDB0000317)

Source

Endogenous

Endogenous (HMDB: HMDB0000317)

Animal

Animal (HMDB: HMDB0000317)

Exogenous

Food

Food (HMDB: HMDB0000317)

Animal origin

Poultry

Pheasant (FooDB: FOOD00430)
Squab (FooDB: FOOD00481)
Turkey (FooDB: FOOD00494)
Quail (FooDB: FOOD00541)
Anatidae (FooDB: FOOD00576)
Rock ptarmigan (FooDB: FOOD00597)
Columbidae (Dove, Pigeon) (FooDB: FOOD00610)
Chicken (FooDB: FOOD00329)
Mallard duck (FooDB: FOOD00353)
Emu (FooDB: FOOD00360)
Greylag goose (FooDB: FOOD00368)
Guinea hen (FooDB: FOOD00373)
Ostrich (FooDB: FOOD00422)
Velvet duck (FooDB: FOOD00427)

Lagomorph

Ovis

Sheep (Mutton, Lamb) (FooDB: FOOD00473)

Bovine

Venison

Deer (FooDB: FOOD00524)
Mule deer (FooDB: FOOD00348)
Elk (FooDB: FOOD00359)

Caprae

Domestic goat (FooDB: FOOD00529)

Swine

Domestic pig (FooDB: FOOD00536)
Wild boar (FooDB: FOOD00307)

Equine

Horse (FooDB: FOOD00378)

Exogenous (HMDB: HMDB0000317)

Process

Naturally occurring process

Biological process

Chemical reaction

Lipid Peroxidation (HMDB: HMDB0000317)

Biochemical pathway

Metabolic pathway

Fatty Acid Metabolism (HMDB: HMDB0000317)

Lipid metabolism pathway (HMDB: HMDB0000317)

Cellular process

Cell signaling (HMDB: HMDB0000317)

Biochemical process

Lipid transport (HMDB: HMDB0000317)

Role

Biological role

Energy source (HMDB: HMDB0000317)

Industrial application

Manufacturing industry

Material processing agent

Fluid processing agent

Surfactant

Surfactant (HMDB: HMDB0000317)

Physical Properties

State

Solid

Experimental Molecular Properties

Property	Value	Reference
Melting Point	Not Available	Not Available
Boiling Point	Not Available	Not Available
Water Solubility	Not Available	Not Available
LogP	Not Available	Not Available

Experimental Chromatographic Properties

Not Available

Predicted Molecular Properties

Property	Value	Source
Water Solubility	149 g/L	ALOGPS
logP	0.74	ALOGPS
logP	0.86	ChemAxon
logS	0.05	ALOGPS
pKa (Strongest Acidic)	4.25	ChemAxon
pKa (Strongest Basic)	-3.9	ChemAxon
Physiological Charge	-1	ChemAxon
Hydrogen Acceptor Count	3	ChemAxon
Hydrogen Donor Count	2	ChemAxon
Polar Surface Area	57.53 Å²	ChemAxon
Rotatable Bond Count	3	ChemAxon
Refractivity	32.44 m³·mol⁻¹	ChemAxon
Polarizability	13.77 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 Chromatographic Properties

Predicted Collision Cross Sections

Predictor	Adduct Type	CCS Value (Å²)	Reference
DarkChem	[M+H]+	129.804	31661259
DarkChem	[M-H]-	124.952	31661259
AllCCS	[M+H]+	132.49	32859911
AllCCS	[M-H]-	128.93	32859911
DeepCCS	[M+H]+	129.776	30932474
DeepCCS	[M-H]-	126.254	30932474
DeepCCS	[M-2H]-	163.423	30932474
DeepCCS	[M+Na]+	138.908	30932474
AllCCS	[M+H]+	132.5	32859911
AllCCS	[M+H-H2O]+	128.4	32859911
AllCCS	[M+NH4]+	136.3	32859911
AllCCS	[M+Na]+	137.5	32859911
AllCCS	[M-H]-	128.9	32859911
AllCCS	[M+Na-2H]-	131.7	32859911
AllCCS	[M+HCOO]-	134.8	32859911

Predicted Kovats Retention Indices

Underivatized

Metabolite	SMILES	Kovats RI Value	Column Type	Reference
2-Hydroxy-3-methylpentanoic acid	CC[C@@H](C)[C@@H](O)C(O)=O	2006.4	Standard polar	33892256
2-Hydroxy-3-methylpentanoic acid	CC[C@@H](C)[C@@H](O)C(O)=O	1083.2	Standard non polar	33892256
2-Hydroxy-3-methylpentanoic acid	CC[C@@H](C)[C@@H](O)C(O)=O	1153.6	Semi standard non polar	33892256

Derivatized

Derivative Name / Structure	SMILES	Kovats RI Value	Column Type	Reference
2-Hydroxy-3-methylpentanoic acid,1TMS,isomer #1	CC[C@@H](C)[C@@H](O[Si](C)(C)C)C(=O)O	1208.1	Semi standard non polar	33892256
2-Hydroxy-3-methylpentanoic acid,1TMS,isomer #2	CC[C@@H](C)[C@@H](O)C(=O)O[Si](C)(C)C	1143.4	Semi standard non polar	33892256
2-Hydroxy-3-methylpentanoic acid,2TMS,isomer #1	CC[C@@H](C)[C@@H](O[Si](C)(C)C)C(=O)O[Si](C)(C)C	1259.7	Semi standard non polar	33892256
2-Hydroxy-3-methylpentanoic acid,1TBDMS,isomer #1	CC[C@@H](C)[C@@H](O[Si](C)(C)C(C)(C)C)C(=O)O	1443.3	Semi standard non polar	33892256
2-Hydroxy-3-methylpentanoic acid,1TBDMS,isomer #2	CC[C@@H](C)[C@@H](O)C(=O)O[Si](C)(C)C(C)(C)C	1364.2	Semi standard non polar	33892256
2-Hydroxy-3-methylpentanoic acid,2TBDMS,isomer #1	CC[C@@H](C)[C@@H](O[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C	1708.3	Semi standard non polar	33892256

Spectra

GC-MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Predicted GC-MS	Predicted GC-MS Spectrum - 2-Hydroxy-3-methylpentanoic acid GC-MS (Non-derivatized) - 70eV, Positive	splash10-004i-9000000000-ed210d81f7dd591ba305	2016-09-22	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - 2-Hydroxy-3-methylpentanoic acid GC-MS (2 TMS) - 70eV, Positive	splash10-070i-9430000000-6a69d25573a59e501509	2017-10-06	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - 2-Hydroxy-3-methylpentanoic acid GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - 2-Hydroxy-3-methylpentanoic acid GC-MS (TMS_1_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - 2-Hydroxy-3-methylpentanoic acid GC-MS (TMS_1_2) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - 2-Hydroxy-3-methylpentanoic acid GC-MS (TBDMS_1_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - 2-Hydroxy-3-methylpentanoic acid GC-MS (TBDMS_1_2) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - 2-Hydroxy-3-methylpentanoic acid GC-MS (TBDMS_2_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum

MS/MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Experimental LC-MS/MS	LC-MS/MS Spectrum - 2-Hydroxy-3-methylpentanoic acid 40V, Negative-QTOF	splash10-0006-9000000000-ee593b4e36128495adaa	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - 2-Hydroxy-3-methylpentanoic acid 20V, Negative-QTOF	splash10-000i-9000000000-dbb7417a40039e144182	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - 2-Hydroxy-3-methylpentanoic acid 10V, Negative-QTOF	splash10-0019-9500000000-af6ad46fd2217e11644c	2021-09-20	HMDB team, MONA	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 2-Hydroxy-3-methylpentanoic acid 10V, Positive-QTOF	splash10-015i-6900000000-a4c1daf19028c3b5134f	2015-09-15	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 2-Hydroxy-3-methylpentanoic acid 20V, Positive-QTOF	splash10-0a4r-9100000000-869a6d71fe88a292f540	2015-09-15	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 2-Hydroxy-3-methylpentanoic acid 40V, Positive-QTOF	splash10-0a4i-9000000000-b1a7373d307d666ee4d4	2015-09-15	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 2-Hydroxy-3-methylpentanoic acid 10V, Negative-QTOF	splash10-001r-6900000000-f3e353eb7493baf38742	2015-09-15	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 2-Hydroxy-3-methylpentanoic acid 20V, Negative-QTOF	splash10-01q0-9500000000-7d4a297dfcfc8042262c	2015-09-15	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 2-Hydroxy-3-methylpentanoic acid 40V, Negative-QTOF	splash10-0a4i-9000000000-befdfe1bd9ab81fcab68	2015-09-15	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 2-Hydroxy-3-methylpentanoic acid 10V, Positive-QTOF	splash10-066r-9200000000-aa3018de5b1cc18a62c9	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 2-Hydroxy-3-methylpentanoic acid 20V, Positive-QTOF	splash10-066s-9000000000-335c8d3f561c609e78a3	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 2-Hydroxy-3-methylpentanoic acid 40V, Positive-QTOF	splash10-0a4l-9000000000-3aa61810dec2a5384f29	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 2-Hydroxy-3-methylpentanoic acid 10V, Negative-QTOF	splash10-001i-0900000000-6a76fa84654b3400f5b4	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 2-Hydroxy-3-methylpentanoic acid 20V, Negative-QTOF	splash10-001i-2900000000-a2221076b3b27d49107e	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 2-Hydroxy-3-methylpentanoic acid 40V, Negative-QTOF	splash10-0a4i-9200000000-02760c89b2616afc5434	2021-09-24	Wishart Lab	View Spectrum

NMR Spectra

Spectrum Type	Description	Deposition Date	Source	View
Predicted 1D NMR	¹³C NMR Spectrum (1D, 100 MHz, H₂O, predicted)	2022-08-23	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 100 MHz, H₂O, predicted)	2022-08-23	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, H₂O, predicted)	2022-08-23	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 1000 MHz, H₂O, predicted)	2022-08-23	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 200 MHz, H₂O, predicted)	2022-08-23	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 200 MHz, H₂O, predicted)	2022-08-23	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 300 MHz, H₂O, predicted)	2022-08-23	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 300 MHz, H₂O, predicted)	2022-08-23	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 400 MHz, H₂O, predicted)	2022-08-23	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 400 MHz, H₂O, predicted)	2022-08-23	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 500 MHz, H₂O, predicted)	2022-08-23	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 500 MHz, H₂O, predicted)	2022-08-23	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 600 MHz, H₂O, predicted)	2022-08-23	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 600 MHz, H₂O, predicted)	2022-08-23	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 700 MHz, H₂O, predicted)	2022-08-23	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 700 MHz, H₂O, predicted)	2022-08-23	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 800 MHz, H₂O, predicted)	2022-08-23	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 800 MHz, H₂O, predicted)	2022-08-23	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 900 MHz, H₂O, predicted)	2022-08-23	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 900 MHz, H₂O, predicted)	2022-08-23	Wishart Lab	View Spectrum

IR Spectra

Spectrum Type	Description	Deposition Date	Source	View
Predicted IR Spectrum	IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M-H]-)	2023-02-03	FELIX lab	View Spectrum
Predicted IR Spectrum	IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+H]+)	2023-02-03	FELIX lab	View Spectrum
Predicted IR Spectrum	IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+Na]+)	2023-02-03	FELIX lab	View Spectrum

Biological Properties

Cellular Locations

Extracellular
Membrane

Biospecimen Locations

Blood
Cerebrospinal Fluid (CSF)
Feces
Saliva
Urine

Tissue Locations

Not Available

Pathways

Not Available

Name	SMPDB/PathBank	KEGG

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	<0.7 uM	Newborn (0-30 days old)	Not Specified	Normal	6422161	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	<0.7 uM	Newborn (0-30 days old)	Not Specified	Normal	6422161	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	28842642	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
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 but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Urine	Detected and Quantified	6.531 +/- 5.519 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Analysis of 30 no...	details
Urine	Detected and Quantified	0.04 (0.02-0.06) umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	Analysis of 40 NI...	details
Urine	Detected and Quantified	0.0 umol/mmol creatinine	Children (1 - 18 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	3.9 (1.7-7.9) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	46.6 uM	Newborn (0-30 days old)	Female	Maple syrup urine disease	6422161	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	17 uM	Newborn (0-30 days old)	Female	Maple syrup urine disease	6422161	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal Cancer	27275383	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	Colorectal cancer	25037050	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Ulcerative colitis	28842642	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Tooth Decay	31026179	details
Urine	Detected and Quantified	13.765 +/- 12.068 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Analysis of 30 no...	details
Urine	Detected but not Quantified	Not Quantified	Not Available	Not Available	Dihydrolipoyl DH (E3) deficiency	11978597	details
Urine	Detected but not Quantified	Not Quantified	Not Available	Not Available	Maple syrup urine disease (MSUD)	11978597	details

Associated Disorders and Diseases

Disease References

Colorectal cancer
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 ] 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 ]
Crohn's disease
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
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 ]
Tooth Decay
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 ]
Maple syrup urine disease
Kumps A, Duez P, Mardens Y: Metabolic, nutritional, iatrogenic, and artifactual sources of urinary organic acids: a comprehensive table. Clin Chem. 2002 May;48(5):708-17. [PubMed:11978597 ] Shigematsu Y, Kikuchi K, Momoi T, Sudo M, Kikawa Y, Nosaka K, Kuriyama M, Haruki S, Sanada K, Hamano N, et al.: Organic acids and branched-chain amino acids in body fluids before and after multiple exchange transfusions in maple syrup urine disease. J Inherit Metab Dis. 1983;6(4):183-9. [PubMed:6422161 ]
Dihydrolipoamide Dehydrogenase Deficiency
Kumps A, Duez P, Mardens Y: Metabolic, nutritional, iatrogenic, and artifactual sources of urinary organic acids: a comprehensive table. Clin Chem. 2002 May;48(5):708-17. [PubMed:11978597 ]
Eosinophilic esophagitis
Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.

Associated OMIM IDs

114500 (Colorectal cancer)
266600 (Crohn's disease)
248600 (Maple syrup urine disease)
246900 (Dihydrolipoamide Dehydrogenase Deficiency)
610247 (Eosinophilic esophagitis)

External Links

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

FDB021944

KNApSAcK ID

C00052119

Chemspider ID

8972081

KEGG Compound ID

Not Available

BioCyc ID

Not Available

BiGG ID

Not Available

Wikipedia Link

Not Available

METLIN ID

5306

PubChem Compound

10796774

PDB ID

Not Available

ChEBI ID

89228

Food Biomarker Ontology

Not Available

VMH ID

Not Available

MarkerDB ID

Not Available

Good Scents ID

Not Available

References

Synthesis Reference

Mamer, O.A. & Reimer, M.L.J. "On the Mechanisms of the Formation of L-Alloisoleucine and the 2-Hydroxy-3-methylvaleric Acid.." J. Biol. Chem. 267: 22141-22147 (1992).

Material Safety Data Sheet (MSDS)

Not Available

General References

Mamer OA, Reimer ML: On the mechanisms of the formation of L-alloisoleucine and the 2-hydroxy-3-methylvaleric acid stereoisomers from L-isoleucine in maple syrup urine disease patients and in normal humans. J Biol Chem. 1992 Nov 5;267(31):22141-7. [PubMed:1429566 ]

Showing metabocard for 2-Hydroxy-3-methylpentanoic acid (HMDB0000317)

MOL for HMDB0000317 (2-Hydroxy-3-methylpentanoic acid)

3D MOL for HMDB0000317 (2-Hydroxy-3-methylpentanoic acid)

3D SDF for HMDB0000317 (2-Hydroxy-3-methylpentanoic acid)

3D-SDF for HMDB0000317 (2-Hydroxy-3-methylpentanoic acid)

PDB for HMDB0000317 (2-Hydroxy-3-methylpentanoic acid)

3D PDB for HMDB0000317 (2-Hydroxy-3-methylpentanoic acid)

SMILES for HMDB0000317 (2-Hydroxy-3-methylpentanoic acid)

INCHI for HMDB0000317 (2-Hydroxy-3-methylpentanoic acid)

Structure for HMDB0000317 (2-Hydroxy-3-methylpentanoic acid)

3D Structure for HMDB0000317 (2-Hydroxy-3-methylpentanoic acid)

Predicted Collision Cross Sections

Predicted Kovats Retention Indices

Underivatized

Derivatized

GC-MS Spectra

MS/MS Spectra

NMR Spectra

IR Spectra