MiMeDB: Showing metabocard for Leucinic acid (MMDBc0000533)

Record Information

Version

1.0

Status

Detected and Quantified

Creation Date

2020-12-10 18:39:55 UTC

Update Date

2024-10-06 21:45:49 UTC

Metabolite ID

MMDBc0000533

Metabolite Identification

Common Name

Leucinic acid

Description

Leucinic acid, also known as leucic acid, 2-hydroxyisocaproic acid or 2-hydroxy-4-methylvaleric acid, belongs to the class of organic compounds known as hydroxy fatty acids. These are fatty acids in which the chain bears a hydroxyl group. Leucinic acid is a valeric acid derivative having a hydroxy substituent at the 2-position and a methyl substituent at the 4-position. It is an alpha-hydroxy analogue of leucine and a metabolite of the branched-chain amino acid leucine. Leucinic acid is found in all organisms ranging from bacteria to plants to animals. Leucinic acid has been found in a patient with dihydrolipoyl dehydrogenase (DLD) deficiency (PMID: 6688766 ). DLD deficiency is caused by mutations in the DLD gene and is inherited in an autosomal recessive manner. A common feature of dihydrolipoamide dehydrogenase deficiency is a potentially life-threatening buildup of lactic acid in tissues (lactic acidosis), which can cause nausea, vomiting, severe breathing problems, and an abnormal heartbeat. Neurological problems are also common in this condition; the first symptoms in affected infants are often decreased muscle tone (hypotonia) and extreme tiredness (lethargy). As the problems worsen, affected infants can have difficulty feeding, decreased alertness, and seizures. Liver problems can also occur in dihydrolipoamide dehydrogenase deficiency, ranging from an enlarged liver (hepatomegaly) to life-threatening liver failure. In some affected people, liver disease, which can begin anytime from infancy to adulthood, is the primary symptom. Leucinic acid is also present in the urine of patients with short bowel syndrome (PMID: 4018104 )

Structure

MOL SDF PDB SMILES InChI

Synonyms

Value	Source
2-Hydroxyisocaproic acid	ChEBI
2-Hydroxyisohexanoic acid	ChEBI
alpha-Hydroxyisocaproic acid	ChEBI
Leucic acid	ChEBI
2-Hydroxyisocaproate	Generator
2-Hydroxyisohexanoate	Generator
a-Hydroxyisocaproate	Generator
a-Hydroxyisocaproic acid	Generator
alpha-Hydroxyisocaproate	Generator
Α-hydroxyisocaproate	Generator
Α-hydroxyisocaproic acid	Generator
Leucate	Generator
Leucinate	Generator
alpha-Hydroxyisocaproic acid, (R)-isomer	HMDB
alpha-Hydroxyisocaproic acid, calcium (2:1) salt, (S)-isomer	HMDB
alpha-Hydroxyisocaproic acid, monosodium salt	HMDB
alpha-Hydroxyisocaproic acid, (S)-isomer	HMDB
2-Hydroxy-4-methyl-valerate	HMDB
2-Hydroxy-4-methyl-valeric acid	HMDB
2-Hydroxy-4-methylpentanoate	HMDB
2-Hydroxy-4-methylpentanoic acid	HMDB
2-Hydroxy-4-methylvalerate	HMDB
2-Hydroxy-4-methylvaleric acid	HMDB
a-Hydroxy-iso-caproate	HMDB
a-Hydroxy-iso-caproic acid	HMDB
alpha-Hydroxy-iso-caproate	HMDB
alpha-Hydroxy-iso-caproic acid	HMDB
DL-2-Hydroxy-4-methylpentanoate	HMDB
DL-2-Hydroxy-4-methylpentanoic acid	HMDB
DL-2-Hydroxyisocaproate	HMDB
DL-2-Hydroxyisocaproic acid	HMDB
DL-a-Hydroxyisocaproate	HMDB
DL-a-Hydroxyisocaproic acid	HMDB
DL-alpha-Hydroxyisocaproate	HMDB
DL-alpha-Hydroxyisocaproic acid	HMDB
DL-Leucate	HMDB
DL-Leucic acid	HMDB
Leucinic acid	ChEBI

Molecular Formula

C₆H₁₂O₃

Average Mass

132.1577

Monoisotopic Mass

132.07864425

IUPAC Name

Not Available

Traditional Name

Not Available

CAS Registry Number

Not Available

SMILES

Not Available

InChI Identifier

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

InChI Key

LVRFTAZAXQPQHI-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

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

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

2-hydroxy fatty acid (CHEBI:59783 )
branched-chain fatty acid (CHEBI:59783 )
Hydroxy fatty acids (LMFA01050381 )

Functional Ontology

Not Available

Physical Properties

State

Expected Solid

Predicted Properties

Not Available

Spectra

Not Available

Biological Properties

Cellular Locations

Extracellular
Membrane

Biospecimen Locations

Blood
Feces
Urine

Tissue Locations

Not Available

Associated OMIM IDs

114500 (Colorectal cancer)
610247 (Eosinophilic esophagitis)

Human Proteins and Enzymes

Protein ID	Protein Name	Protein Type	Uniprot ID	Species

Human Pathways

Pathways

Name	SMPDB/PathBank

Metabolic Reactions

Reactions

Reaction ID	Precursor	Product	Enzyme	Reaction Type	Data Source	Reference
MMDBr0004219	Leucinic acid	Ketoleucine	Not Available		VMH	6434570 6688766

Health Effects and Bioactivity

Health Outcome/Bioactivity	Metabolite Response/Effect	Evidence Type	Host and Biospecimen	Data Source	Reference

Microbial Sources

Superkingdom/Kingdom	Phylum	Total species	Hosts and Body Sites	Microbial Links
Bacteria	Bacteroidetes	1	Human ; Human feces	Indirect association between microbiota composition and metabolites measured in host biospecimen
Bacteria	Actinobacteria	1	Human ; Human supragingival plaque; Human subgingival plaque; Human feces	Indirect association between microbiota composition and metabolites measured in host biospecimen
Eukaryota/Fungi	Ascomycota	1	Human feces; Human saliva	Unknown

Exposure Sources

Source Type	Activity	Data Source	Reference
Cosmetics	Not Available	U.S. Environmental Protection Agency	CompTox
Domestics	Not Available	U.S. Environmental Protection Agency	CompTox

Host Biospecimen and Location

Host and Biospecimen	Status	Mean	Min	Max	Units	Age	Sex	Health Condition	Data Source	Reference
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	Colorectal cancer	HMDB	25037050
Human Feces	Detected but not Quantified					Adult (>18 years old)	Both	Colorectal cancer	HMDB	27015276
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	29808030
Human Urine	Detected and Quantified		0	50	umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	31506554
Human Urine	Detected and Quantified		0	50	umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	31506554
Human Urine	Detected and Quantified	0			umol/mmol creatinine	Children (1 - 18 years old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab
Human Urine	Detected and Quantified		0	0.39	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	Great Plains Laboratory Reference Values (Males >13)
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
Human Blood	Detected but not Quantified								HMDB

External Links

HMDB ID

HMDB0000665

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

FDB022171

KNApSAcK ID

Not Available

Chemspider ID

83753

KEGG Compound ID

Not Available

BioCyc ID

Not Available

BiGG ID

Not Available

Wikipedia Link

Not Available

METLIN ID

5635

PubChem Compound

92779

PDB ID

Not Available

ChEBI ID

59783

Food Biomarker Ontology

Not Available

References

Synthesis Reference

Not Available

General References

Spaapen LJ, Ketting D, Wadman SK, Bruinvis L, Duran M: Urinary D-4-hydroxyphenyllactate, D-phenyllactate and D-2-hydroxyisocaproate, abnormalities of bacterial origin. J Inherit Metab Dis. 1987;10(4):383-90. doi: 10.1007/BF01799981. [PubMed:3126358 ]
Haan E, Brown G, Bankier A, Mitchell D, Hunt S, Blakey J, Barnes G: Severe illness caused by the products of bacterial metabolism in a child with a short gut. Eur J Pediatr. 1985 May;144(1):63-5. doi: 10.1007/BF00491929. [PubMed:4018104 ]
Jakobs C, Sweetman L, Nyhan WL: Hydroxy acid metabolites of branched-chain amino acids in amniotic fluid. Clin Chim Acta. 1984 Jul 16;140(2):157-66. doi: 10.1016/0009-8981(84)90340-1. [PubMed:6467607 ]
Kuhara T, Shinka T, Inoue Y, Matsumoto M, Yoshino M, Sakaguchi Y, Matsumoto I: Studies of urinary organic acid profiles of a patient with dihydrolipoyl dehydrogenase deficiency. Clin Chim Acta. 1983 Sep 30;133(2):133-40. doi: 10.1016/0009-8981(83)90398-4. [PubMed:6688766 ]

Showing metabocard for Leucinic acid (MMDBc0000533)

MOL for #<Metabolite:0x00007fdb4d16a168>

3D MOL for #<Metabolite:0x00007fdb4d16a168>

3D SDF for #<Metabolite:0x00007fdb4d16a168>

3D-SDF for #<Metabolite:0x00007fdb4d16a168>

PDB for #<Metabolite:0x00007fdb4d16a168>

3D PDB for #<Metabolite:0x00007fdb4d16a168>

SMILES for #<Metabolite:0x00007fdb4d16a168>

INCHI for #<Metabolite:0x00007fdb4d16a168>

Structure for #<Metabolite:0x00007fdb4d16a168>

3D Structure for #<Metabolite:0x00007fdb4d16a168>