MiMeDB: Showing metabocard for 3-Hydroxyisovaleric acid (MMDBc0000538)

Record Information

Version

1.0

Status

Detected and Quantified

Creation Date

2020-12-10 18:40:02 UTC

Update Date

2024-04-30 19:32:53 UTC

Metabolite ID

MMDBc0000538

Metabolite Identification

Common Name

3-Hydroxyisovaleric acid

Description

3-Hydroxyisovaleric acid is a normal human metabolite excreted in the urine. It is a byproduct of the leucine degradation pathway. Production of 3-hydroxyisovaleric acid begins with the conversion of 3-methylcrotonyl-CoA into 3-methylglutaconyl-CoA in the mitochondria by the biotin-dependent enzyme methylcrotonyl-CoA carboxylase. Biotin deficiencies, certain lifestyle habits (smoking), or specific genetic conditions can reduce methylcrotonyl-CoA carboxylase activity. This reduction can lead to a buildup of 3-methylcrotonyl-CoA, which is converted into 3-hydroxyisovaleryl-CoA by the enzyme enoyl-CoA hydratase. Increased concentrations of 3-methylcrotonyl-CoA and 3-hydroxyisovaleryl-CoA can lead to a disruption of the esterified CoA:free CoA ratio, and ultimately to mitochondrial toxicity. Detoxification of these metabolic end products occur via the transfer of the 3-hydroxyisovaleryl moiety to carnitine forming 3-hydroxyisovaleric acid-carnitine or 3HIA-carnitine, which is then transferred across the inner mitochondrial membrane where 3-hydroxyisovaleric acid is released as the free acid (PMID: 21918059 ). 3-Hydroxyisovaleric acid has been found to be elevated in smokers and in subjects undergoing long-term anticonvulsant therapy with carbamazepine and/or phenytoin. These levels are elevated due to impairment of renal reclamation of biotin. Levels may also be increased from prolonged consumption of raw egg-whites (PMID: 16895887 , 9523856 , 15447901 , 9176832 ) (OMIM: 210210 , 253270 , 600529 , 253260 , 246450 , 210200 , 238331 ). When present in sufficiently high levels, 3-hydroxyisovaleric acid can act as an acidogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of 3-hydroxyisovaleric acid are associated with at least a dozen inborn errors of metabolism, including 3-hydroxy-3-methylglutaryl-CoA lyase deficiency, 3-methylglutaconic aciduria type I, biotinidase deficiency and isovaleric aciduria, dihydrolipoamide dehydrogenase deficiency, 3-methylcrotonyl-CoA carboxylase 1 deficiency, 3-hydroxy-3-methylglutaryl-CoA lyase deficiency, late-onset multiple carboxylase deficiency, holocarboxylase synthetase deficiency, and 3-methylcrotonyl-CoA carboxylase 2 deficiency. 3-Hydroxyisovaleric acid is an organic acid. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart, liver, and kidney abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of the untreated IEMs mentioned above. Many affected children with organic acidemias experience intellectual disability or delayed development. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and seizures.

Structure

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

Synonyms

Value	Source
3-Hydroxy-3-methylbutyric acid	ChEBI
3-Hydroxy-isovaleric acid	ChEBI
3-Methyl-3-hydroxybutyric acid	ChEBI
3-OH-Isovaleric acid	ChEBI
beta-Hydroxy-beta-methylbutyric acid	ChEBI
beta-Hydroxyisovaleric acid	ChEBI
HMB	ChEBI
HMB-D6	ChEBI
3-Hydroxy-3-methylbutyrate	Generator
3-Hydroxy-isovalerate	Generator
3-Methyl-3-hydroxybutyrate	Generator
3-OH-Isovalerate	Generator
b-Hydroxy-b-methylbutyrate	Generator
b-Hydroxy-b-methylbutyric acid	Generator
beta-Hydroxy-beta-methylbutyrate	Generator
Β-hydroxy-β-methylbutyrate	Generator
Β-hydroxy-β-methylbutyric acid	Generator
b-Hydroxyisovalerate	Generator
b-Hydroxyisovaleric acid	Generator
beta-Hydroxyisovalerate	Generator
Β-hydroxyisovalerate	Generator
Β-hydroxyisovaleric acid	Generator
3-Hydroxyisovalerate	Generator
beta Hydroxy beta methylbutyrate	HMDB
beta-Hydroxy beta-methylbutyrate	HMDB
3-Hydroxy-3-methyl-butanoate	HMDB
3-Hydroxy-3-methyl-butanoic acid	HMDB
3-Hydroxy-3-methyl-butyric acid	HMDB
3-Hydroxy-3-methylbutanoate	HMDB
3-Hydroxy-3-methylbutanoic acid	HMDB
3-Hydroxyisovaleric acid	MeSH

Molecular Formula

C₅H₁₀O₃

Average Mass

118.1311

Monoisotopic Mass

118.062994186

IUPAC Name

3-hydroxy-3-methylbutanoic acid

Traditional Name

3-hydroxyisovaleric acid

CAS Registry Number

Not Available

SMILES

CC(C)(O)CC(O)=O

InChI Identifier

InChI=1S/C5H10O3/c1-5(2,8)3-4(6)7/h8H,3H2,1-2H3,(H,6,7)

InChI Key

AXFYFNCPONWUHW-UHFFFAOYSA-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

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

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

3-hydroxy monocarboxylic acid (CHEBI:37084 )
Hydroxy fatty acids (LMFA01050396 )

Functional Ontology

Not Available

Physical Properties

State

Expected Solid

Predicted Properties

Property	Value	Source
Water Solubility	384 g/L	ALOGPS
logP	-0.12	ALOGPS
logP	-0.11	ChemAxon
logS	0.51	ALOGPS
pKa (Strongest Acidic)	4.55	ChemAxon
pKa (Strongest Basic)	-2.7	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	2	ChemAxon
Refractivity	28.1 m³·mol⁻¹	ChemAxon
Polarizability	11.75 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

Spectra

Spectrum Type	Description	Splash Key	Deposition Date	View
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)	splash10-000t-0900000000-56f9e1ee9e19bacfc939	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-000t-0900000000-56f9e1ee9e19bacfc939	2017-09-12	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	splash10-0a4i-9100000000-0809a885d114ba6b21d7	2017-09-01	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positive	splash10-0089-6910000000-3573bf97996ef7107955	2017-10-06	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_2) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_2) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_2_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 35V, Negative	splash10-0006-9000000000-1381eacc487b12932a43	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 20V, Negative	splash10-00dj-9000000000-f10c5afd9954f294ea91	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 10V, Negative	splash10-00xr-9600000000-3b24b27c64b89a6a5b25	2021-09-20	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)	splash10-0a4i-9300000000-e715db06e575104deccd	2012-07-24	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)	splash10-0a4i-9000000000-885e3d959712c9998250	2012-07-24	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)	splash10-0avi-9800000000-f0296f537bd22b26f173	2012-07-24	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 35V, Positive	splash10-014i-1900000000-b947237de42eec92a67e	2021-09-20	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Negative	splash10-00xr-9400000000-13e4ec512c0fde3123ab	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Negative	splash10-05tb-9200000000-96c7d7b3f54e7377a875	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Negative	splash10-0aba-9000000000-3cc71227974213e131c4	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Negative	splash10-00kb-9400000000-9721ad435e43092a072d	2021-09-22	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Negative	splash10-0a4i-9000000000-13f5bd4a628f51aec810	2021-09-22	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Negative	splash10-052f-9000000000-0eeab2c474971ae60e7b	2021-09-22	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-0ue9-5900000000-2f664d0b967c87e7358c	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-0zn9-9500000000-233e5cd462b504c09d3c	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-0a4i-9000000000-62361f03b7c7a0d82bc4	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-0udi-8900000000-a25104a0136877edd57e	2021-09-22	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-052f-9000000000-d2d6bcff64e0a706fc95	2021-09-22	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-0006-9000000000-65f5183bc669daba34f4	2021-09-22	View Spectrum
MS	Mass Spectrum (Electron Ionization)	splash10-052f-9000000000-c15a37ad10e6379350aa	2014-09-20	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 600 MHz, H₂O, experimental)		2012-12-04	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 100 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 600 MHz, H₂O, experimental)		2021-10-10	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 600 MHz, H₂O, experimental)		2021-10-10	View Spectrum
2D NMR	[¹H, ¹³C]-HSQC NMR Spectrum (2D, 600 MHz, H₂O, experimental)		2012-12-05	View Spectrum

Biological Properties

Cellular Locations

Cytoplasm

Biospecimen Locations

Blood
Cerebrospinal Fluid (CSF)
Feces
Fibroblasts
Placenta
Urine

Tissue Locations

Fibroblasts
Placenta

Associated OMIM IDs

114500 (Colorectal cancer)
610247 (Eosinophilic esophagitis)

Human Proteins and Enzymes

Protein ID	Protein Name	Protein Type	Uniprot ID	Species
MMDBp0192140	UDP-glucuronosyltransferase 1-1	Enzyme	P22309	Homo sapiens

Human Pathways

Pathways

Name	SMPDB/PathBank

Metabolic Reactions

Reaction ID	Precursor	Product	Enzyme	Reaction Type	Data Source	Reference
MMDBr0003972	Ketoleucine	3-Hydroxyisovaleric acid	Not Available	RE1266C	VMH	11027593
MMDBr0005834	3-Hydroxyisovaleric acid	3-Hydroxyisovaleric acid	Not Available	Transport for Beta-Hydroxy-Beta-Methylbutyrate	VMH	30371894
MMDBr0008622	3-Hydroxyisovaleryl-CoA	3-Hydroxyisovaleric acid	Not Available	Formation of 3-Hydroxy-Isovalerate	VMH	30371894
MMDBr0008623	3-Hydroxyisovaleric acid	3-Hydroxyisovaleric acid	Not Available	Transport of 3-Hydroxy-Isovalerate, Mitochondrial	VMH	30371894
MMDBr0008624	3-Hydroxyisovaleric acid	3-Hydroxyisovaleric acid	Not Available	Transport of 3-Hydroxy-Isovalerate, Extracellular	VMH	30371894
MMDBr0038752	3-Hydroxyisovaleryl-CoA	3-Hydroxyisovaleric acid	Thioesterase TesA	Hydrolysis	RHEA	34755880

Health Effects and Bioactivity

Health Outcome/Bioactivity	Metabolite Response/Effect	Evidence Type	Host and Biospecimen	Data Source	Reference
3-hydroxy-3-methylglutaryl-coa lyase deficiency	Increased	Association	Human Urine	HMDB	15505778
3-hydroxy-3-methylglutaryl-coa lyase deficiency		Association	Human Urine	HMDB	23705938
3-methyl-crotonyl-glycinuria		Association	Human Cerebrospinal Fluid (CSF)	HMDB	11893004
3-methyl-crotonyl-glycinuria	Increased	Association	Human Urine	HMDB	11893004
3-methyl-crotonyl-glycinuria	Increased	Association	Human Urine	HMDB	25732994
3-methylglutaconic aciduria type i	Increased	Association	Human Blood	HMDB	16541463
3-methylglutaconic aciduria type i	Increased	Association	Human Cerebrospinal Fluid (CSF)	HMDB	16541463
Lung cancer	Slight Decrease	Association	Human Urine	HMDB	18953024
Eosinophilic esophagitis		Association	Human Urine	HMDB	Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Eosinophilic esophagitis		Association	Human Urine	HMDB	Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Diabetes mellitus type 1		Association	Human Urine	HMDB	Lorena Ivona ŞTEFAN, Alina NICOLESCU, Simona POPA, Maria MOŢA, Eugenia KOVACS and Calin DELEANU. 1H-NMR URINE METABOLIC PROFILING IN TYPE 1 DIABETES MELLITUS. Rev. Roum. Chim., 2010, 55(11-12), 1033-1037
3-hydroxy-3-methylglutaryl-coa lyase deficiency	Increased	Association	Human Urine	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
3-hydroxy-3-methylglutaryl-coa lyase deficiency		Association	Human Urine	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Biotinidase deficiency	Increased	Association	Human Urine	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Biotinidase deficiency		Association	Human Blood	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Biotinidase deficiency		Association	Human Blood	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)

Microbial Sources

Superkingdom/Kingdom	Phylum	Total species	Hosts and Body Sites	Microbial Links
Bacteria	Firmicutes	5	Human stool; Human ; Human feces	Indirect association between microbiota composition and metabolites measured in host biospecimen
Archaea	Euryarchaeota	1	Human feces; Human	Indirect association between microbiota composition and metabolites measured in host biospecimen

Exposure Sources

Source Type	Source Sub-type	Species	Data Source	Reference
Plant	Fruit juice	Vaccinium myrtillus	FooDB	31437929

Host Biospecimen and Location

Host and Biospecimen	Status	Mean	Std	Min	Max	Units	Age	Sex	Health Condition	Data Source	Reference
Human Feces	Detected but not Quantified									HMDB
Human Urine	Detected and Quantified	851				umol/mmol creatinine	Adult (>18 years old)	Female	3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency	HMDB	15505778
Human Urine	Detected and Quantified	851				umol/mmol creatinine	Adult (>18 years old)	Female	3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency	HMDB	15505778
Human Urine	Detected and Quantified	1154.8				umol/mmol creatinine	Newborn (0-30 days old)	Male	3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency	HMDB	23705938
Human Urine	Detected and Quantified	1154.8				umol/mmol creatinine	Newborn (0-30 days old)	Male	3-hydroxy-3-methylglutaric- coenzyme A lyase deficiency	HMDB	23705938
Human Cerebrospinal fluid (csf)	Detected and Quantified	12				uM	Children (1 - 13 years old)	Not Specified	Isolated 3-Methylcrotonyl-CoA Carboxylase Deficiency	HMDB	11893004
Human Urine	Detected and Quantified	2974				umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Isolated 3-Methylcrotonyl-CoA Carboxylase Deficiency	HMDB	11893004
Human Urine	Detected and Quantified	1900	500			umol/mmol creatinine	Adult (>18 years old)	Both	3-Methylcrotonyl-CoA Carboxylase Deficiency	HMDB	25732994
Human Blood	Detected and Quantified	20				uM	Adult (>18 years old)	Female	3-methylglutaconic aciduria type I	HMDB	16541463
Human Cerebrospinal fluid (csf)	Detected and Quantified	172				uM	Adult (>18 years old)	Female	3-methylglutaconic aciduria type I	HMDB	16541463
Human Blood	Detected and Quantified	10.0		10.0	20.0	uM	Adult (>18 years old)	Both	3-Methylglutaconic aciduria type I	HMDB	16541463
Human Urine	Detected and Quantified			0.0100	0.0460	umol/mmol creatinine	Children (1-13 years old)	Female	3-Methylglutaconic Aciduria (Type VI)	HMDB	16527507
Human Blood	Detected and Quantified	9.2	1.7			uM	Adult (>18 years old)	Female	Early preeclampsia	HMDB	22494326
Human Blood	Detected and Quantified	6.5	3.3			uM	Adult (>18 years old)	Female	Late-onset preeclampsia	HMDB	23159745
Human Urine	Detected and Quantified	6.0	2.0			umol/mmol creatinine	Adult (>18 years old)	Both	Lung cancer	HMDB	18953024
Human Blood	Detected and Quantified	4.7	2.5			uM	Adult (>18 years old)	Female	Pregnancy	HMDB	23159745
Human Blood	Detected and Quantified	5.24	3.84			uM	Adult (>18 years old)	Female	Pregnancy	HMDB	23535240
Human Blood	Detected and Quantified	7.6	3.1			uM	Adult (>18 years old)	Female	Pregnancy	HMDB	22494326
Human Urine	Detected and Quantified	<0.0420				umol/mmol creatinine	Not Specified	Not Specified	Normal	HMDB	16527507
Human Blood	Detected and Quantified	<10				uM	Adult (>18 years old)	Female	Normal	HMDB	16541463
Human Cerebrospinal fluid (csf)	Detected and Quantified	<10				uM	Adult (>18 years old)	Female	Normal	HMDB	16541463
Human Urine	Detected and Quantified	<46				umol/mmol creatinine	Not Specified	Not Specified	Normal	HMDB	23705938
Human Urine	Detected and Quantified	<50				umol/mmol creatinine	Children (1 - 13 years old)	Female	Normal	HMDB	2587127
Human Urine	Detected and Quantified	<50				umol/mmol creatinine	Adult (>18 years old)	Female	Normal	HMDB	15505778
Human Urine	Detected and Quantified	<50				umol/mmol creatinine	Not Specified	Not Specified	Normal	HMDB	15505778
Human Urine	Detected and Quantified			0	1.3	umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	HMDB	31963255
Human Urine	Detected and Quantified			0	1.3	umol/mmol creatinine	Infant (0-1 year old)	Female	Normal	HMDB	31963255
Human Urine	Detected and Quantified			0.0	3.2	umol/mmol creatinine	Infant (0-1 year old)	Female	Normal	HMDB	31963255
Human Urine	Detected and Quantified	11		6.9	25	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	2702744
Human Urine	Detected and Quantified	12.2		0.1	43.2	umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	8087979
Human Urine	Detected and Quantified	26.9		9.1	66.4	umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	HMDB	8087979
Human Blood	Detected and Quantified	4.0		0.0	10.0	uM	Adult (>18 years old)	Not Specified	Normal	HMDB	16541463
Human Cerebrospinal fluid (csf)	Detected and Quantified	4.0	2.0			uM	Adult (>18 years old)	Both	Normal	HMDB	18502700
Human Urine	Detected and Quantified			4.3	5.7	umol/mmol creatinine	Adult (>18 years old)	Female	Normal	HMDB	27012787
Human Urine	Detected and Quantified			4.5	6.3	umol/mmol creatinine	Adult (>18 years old)	Male	Normal	HMDB	27012787
Human Urine	Detected and Quantified	6	4			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	HMDB	11893004
Human Urine	Detected and Quantified	6.8		3.2	21.8	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Urine	Detected and Quantified	7.4		4.1	17.2	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Urine	Detected and Quantified	7.5		5.1	23.1	umol/mmol creatinine	Children (1-13 years old)	Both	Normal	HMDB	8087979
Human Urine	Detected and Quantified	8.5	3.2			umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	21892638
Human Urine	Detected and Quantified	9.0		3.1	17.4	umol/mmol creatinine	Adolescent (13-18 years old)	Both	Normal	HMDB	8087979
Human Urine	Detected and Quantified	12.757	10.313			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified	247.428	452.008			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified	10.78	4.94 ‚Äì 21.07			umol/mmol creatinine	Adult (>18 years old)	Both	Type 1 diabetes Mellitus	HMDB	Lorena Ivona ŞTEFAN, Alina NICOLESCU, Simona POPA, Maria MOŢA, Eugenia KOVACS and Calin DELEANU. 1H-NMR URINE METABOLIC PROFILING IN TYPE 1 DIABETES MELLITUS. Rev. Roum. Chim., 2010, 55(11-12), 1033-1037
Human Urine	Detected and Quantified	2030.0		60.0	4000.0	umol/mmol creatinine	Children (1-13 years old)	Both	3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Urine	Detected and Quantified	23.0		0.0	46.0	umol/mmol creatinine	Adult (>18 years old)	Both	3-Hydroxy-3-methylglutaryl-CoA lyase deficiency	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Urine	Detected and Quantified	275.0		50.0	500.0	umol/mmol creatinine	Children (1-13 years old)	Both	Biotinidase deficiency	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Blood	Detected and Quantified	88.0		10.0	166.0	uM	Children (1-13 years old)	Both	Biotinidase deficiency	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Blood	Detected and Quantified	9.5		0.00	19.00	uM	Adult (>18 years old)	Both	Biotinidase deficiency	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Urine	Detected and Quantified	11.427	7.8			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified	40.486	23.766			umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified	<68				umol/mmol creatinine	Children (1 - 18 years old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab
Human Urine	Detected and Quantified	15.0		5.0	26.0	umol/mmol creatinine	Children (1-13 years old)	Both	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Human Urine	Detected and Quantified	2.0		0.0	4.0	umol/mmol creatinine	Children (1-13 years old)	Both	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Human Urine	Detected and Quantified	10.50	7.02 ‚Äì 14.38			umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	Lorena Ivona ŞTEFAN, Alina NICOLESCU, Simona POPA, Maria MOŢA, Eugenia KOVACS and Calin DELEANU. 1H-NMR URINE METABOLIC PROFILING IN TYPE 1 DIABETES MELLITUS. Rev. Roum. Chim., 2010, 55(11-12), 1033-1037
Human Urine	Detected and Quantified	3.56				umol/mmol creatinine	Adult (>18 years old)	Male	Normal	HMDB	Shaykhutdinov RA, MacInnis GD, Dowlatabadi R, Weljie AM, Vogel HJ. Quantitative analysis of metabolite concentrations in human urine samples using 13C{1H} NMR spectroscopy. Metabolomics. 2009
Human Fibroblasts	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Placenta	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Urine	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Urine	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22932811
Human Urine	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	21389975
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	18085514
Human Blood	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	21505807
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	27065191
Human Blood	Detected but not Quantified						Adult (>18 years old)	Female	Normal	HMDB	27355821
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	27543620
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	25037050
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	29808030

External Links

HMDB ID

HMDB0000754

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

FDB022225

KNApSAcK ID

Not Available

Chemspider ID

62571

KEGG Compound ID

Not Available

BioCyc ID

Not Available

BiGG ID

Not Available

Wikipedia Link

Beta-Hydroxy_beta-methylbutyric_acid

METLIN ID

5722

PubChem Compound

69362

PDB ID

Not Available

ChEBI ID

37084

Food Biomarker Ontology

Not Available

References

Synthesis Reference

Not Available

General References

Jakobs C, Sweetman L, Nyhan WL, Packman S: Stable isotope dilution analysis of 3-hydroxyisovaleric acid in amniotic fluid: contribution to the prenatal diagnosis of inherited disorders of leucine catabolism. J Inherit Metab Dis. 1984;7(1):15-20. doi: 10.1007/BF01805614. [PubMed:6429435 ]
Guneral F, Bachmann C: Age-related reference values for urinary organic acids in a healthy Turkish pediatric population. Clin Chem. 1994 Jun;40(6):862-6. [PubMed:8087979 ]
Mock DM, Stadler DD: Conflicting indicators of biotin status from a cross-sectional study of normal pregnancy. J Am Coll Nutr. 1997 Jun;16(3):252-7. doi: 10.1080/07315724.1997.10718682. [PubMed:9176832 ]
Schurmann M, Engelbrecht V, Lohmeier K, Lenard HG, Wendel U, Gartner J: Cerebral metabolic changes in biotinidase deficiency. J Inherit Metab Dis. 1997 Nov;20(6):755-60. doi: 10.1023/a:1005307415289. [PubMed:9427142 ]
Mock DM, Mock NI, Nelson RP, Lombard KA: Disturbances in biotin metabolism in children undergoing long-term anticonvulsant therapy. J Pediatr Gastroenterol Nutr. 1998 Mar;26(3):245-50. doi: 10.1097/00005176-199803000-00002. [PubMed:9523856 ]
Amberg A, Rosner E, Dekant W: Biotransformation and kinetics of excretion of tert-amyl-methyl ether in humans and rats after inhalation exposure. Toxicol Sci. 2000 Jun;55(2):274-83. doi: 10.1093/toxsci/55.2.274. [PubMed:10828258 ]
Santer R, Muhle H, Suormala T, Baumgartner ER, Duran M, Yang X, Aoki Y, Suzuki Y, Stephani U: Partial response to biotin therapy in a patient with holocarboxylase synthetase deficiency: clinical, biochemical, and molecular genetic aspects. Mol Genet Metab. 2003 Jul;79(3):160-6. doi: 10.1016/s1096-7192(03)00091-x. [PubMed:12855220 ]
Rodriguez JM, Ruiz-Sala P, Ugarte M, Penalva MA: Fungal metabolic model for 3-methylcrotonyl-CoA carboxylase deficiency. J Biol Chem. 2004 Feb 6;279(6):4578-87. doi: 10.1074/jbc.M310055200. Epub 2003 Nov 11. [PubMed:14612443 ]
Boulat O, Gradwohl M, Matos V, Guignard JP, Bachmann C: Organic acids in the second morning urine in a healthy Swiss paediatric population. Clin Chem Lab Med. 2003 Dec;41(12):1642-58. doi: 10.1515/CCLM.2003.248. [PubMed:14708889 ]
Sealey WM, Teague AM, Stratton SL, Mock DM: Smoking accelerates biotin catabolism in women. Am J Clin Nutr. 2004 Oct;80(4):932-5. doi: 10.1093/ajcn/80.4.932. [PubMed:15447901 ]
Baykal T, Gokcay GH, Ince Z, Dantas MF, Fowler B, Baumgartner MR, Demir F, Can G, Demirkol M: Consanguineous 3-methylcrotonyl-CoA carboxylase deficiency: early-onset necrotizing encephalopathy with lethal outcome. J Inherit Metab Dis. 2005;28(2):229-33. doi: 10.1007/s10545-005-4559-8. [PubMed:15877210 ]
Stratton SL, Bogusiewicz A, Mock MM, Mock NI, Wells AM, Mock DM: Lymphocyte propionyl-CoA carboxylase and its activation by biotin are sensitive indicators of marginal biotin deficiency in humans. Am J Clin Nutr. 2006 Aug;84(2):384-8. doi: 10.1093/ajcn/84.1.384. [PubMed:16895887 ]
Mock DM, Stratton SL, Horvath TD, Bogusiewicz A, Matthews NI, Henrich CL, Dawson AM, Spencer HJ, Owen SN, Boysen G, Moran JH: Urinary excretion of 3-hydroxyisovaleric acid and 3-hydroxyisovaleryl carnitine increases in response to a leucine challenge in marginally biotin-deficient humans. J Nutr. 2011 Nov;141(11):1925-30. doi: 10.3945/jn.111.146126. Epub 2011 Sep 14. [PubMed:21918059 ]
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 ]

Showing metabocard for 3-Hydroxyisovaleric acid (MMDBc0000538)

MOL for #<Metabolite:0x00007fe26b36d580>

3D MOL for #<Metabolite:0x00007fe26b36d580>

3D SDF for #<Metabolite:0x00007fe26b36d580>

3D-SDF for #<Metabolite:0x00007fe26b36d580>

PDB for #<Metabolite:0x00007fe26b36d580>

3D PDB for #<Metabolite:0x00007fe26b36d580>

SMILES for #<Metabolite:0x00007fe26b36d580>

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Structure for #<Metabolite:0x00007fe26b36d580>

3D Structure for #<Metabolite:0x00007fe26b36d580>