GlyCosmos Portal

Annotation

Keyword

Acetylation
Alternative splicing
Disease variant
Intrahepatic cholestasis
Isomerase
Mitochondrion
Peroxisome
Reference proteome

Proteins

Displaying 1 entry
UniProt	Protein Name
Q9UHK6	2-methylacyl-CoA racemase

Annotation information from UniProt.

Gene Ontology (GO)

Displaying **all 5** entries
GO Term	Evidence Code	PMID
bile acid biosynthetic process	TAS
bile acid biosynthetic process	IEA
bile acid metabolic process	IDA	10655068
bile acid metabolic process	IBA
fatty acid beta-oxidation using acyl-CoA oxidase	TAS

GO Hierarchy

biological process

metabolic process [inference]

catabolic process [inference]

cellular catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

cellular lipid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

small molecule catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

organic substance catabolic process [inference]

lipid catabolic process [inference]

cellular lipid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

biosynthetic process [inference]

cellular biosynthetic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

bile acid biosynthetic process

small molecule biosynthetic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

bile acid biosynthetic process

organic substance biosynthetic process [inference]

lipid biosynthetic process [inference]

steroid biosynthetic process [inference]

bile acid biosynthetic process

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

bile acid biosynthetic process

organic cyclic compound biosynthetic process [inference]

steroid biosynthetic process [inference]

bile acid biosynthetic process

organic hydroxy compound biosynthetic process [inference]

bile acid biosynthetic process

cellular metabolic process [inference]

organic acid metabolic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

bile acid biosynthetic process

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

monocarboxylic acid metabolic process [inference]

fatty acid metabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

fatty acid oxidation [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

bile acid metabolic process

bile acid biosynthetic process

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

monocarboxylic acid biosynthetic process [inference]

bile acid biosynthetic process

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

bile acid biosynthetic process

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

cellular catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

cellular lipid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

cellular biosynthetic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

bile acid biosynthetic process

cellular lipid metabolic process [inference]

fatty acid metabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

fatty acid oxidation [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

lipid modification [inference]

lipid oxidation [inference]

fatty acid oxidation [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

cellular lipid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

primary metabolic process [inference]

lipid metabolic process [inference]

steroid metabolic process [inference]

steroid biosynthetic process [inference]

bile acid biosynthetic process

bile acid metabolic process

bile acid biosynthetic process

lipid biosynthetic process [inference]

steroid biosynthetic process [inference]

bile acid biosynthetic process

lipid catabolic process [inference]

cellular lipid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

cellular lipid metabolic process [inference]

fatty acid metabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

fatty acid oxidation [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

lipid modification [inference]

lipid oxidation [inference]

fatty acid oxidation [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

cellular lipid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

small molecule metabolic process [inference]

organic acid metabolic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

bile acid biosynthetic process

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

monocarboxylic acid metabolic process [inference]

fatty acid metabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

fatty acid oxidation [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

bile acid metabolic process

bile acid biosynthetic process

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

monocarboxylic acid biosynthetic process [inference]

bile acid biosynthetic process

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

bile acid biosynthetic process

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

small molecule catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

small molecule biosynthetic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

bile acid biosynthetic process

organic substance metabolic process [inference]

organic acid metabolic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

bile acid biosynthetic process

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

monocarboxylic acid metabolic process [inference]

fatty acid metabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

fatty acid oxidation [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

bile acid metabolic process

bile acid biosynthetic process

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

monocarboxylic acid biosynthetic process [inference]

bile acid biosynthetic process

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

bile acid biosynthetic process

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

lipid metabolic process [inference]

steroid metabolic process [inference]

steroid biosynthetic process [inference]

bile acid biosynthetic process

bile acid metabolic process

bile acid biosynthetic process

lipid biosynthetic process [inference]

steroid biosynthetic process [inference]

bile acid biosynthetic process

lipid catabolic process [inference]

cellular lipid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

cellular lipid metabolic process [inference]

fatty acid metabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

fatty acid oxidation [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

lipid modification [inference]

lipid oxidation [inference]

fatty acid oxidation [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

cellular lipid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

organic cyclic compound metabolic process [inference]

steroid metabolic process [inference]

steroid biosynthetic process [inference]

bile acid biosynthetic process

bile acid metabolic process

bile acid biosynthetic process

organic cyclic compound biosynthetic process [inference]

steroid biosynthetic process [inference]

bile acid biosynthetic process

organic substance catabolic process [inference]

lipid catabolic process [inference]

cellular lipid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

organic substance biosynthetic process [inference]

lipid biosynthetic process [inference]

steroid biosynthetic process [inference]

bile acid biosynthetic process

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

bile acid biosynthetic process

organic cyclic compound biosynthetic process [inference]

steroid biosynthetic process [inference]

bile acid biosynthetic process

organic hydroxy compound biosynthetic process [inference]

bile acid biosynthetic process

organic hydroxy compound metabolic process [inference]

bile acid metabolic process

bile acid biosynthetic process

organic hydroxy compound biosynthetic process [inference]

bile acid biosynthetic process

cellular process [inference]

cellular metabolic process [inference]

organic acid metabolic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

bile acid biosynthetic process

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

monocarboxylic acid metabolic process [inference]

fatty acid metabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

fatty acid oxidation [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

bile acid metabolic process

bile acid biosynthetic process

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

monocarboxylic acid biosynthetic process [inference]

bile acid biosynthetic process

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

bile acid biosynthetic process

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

cellular catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

cellular lipid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

cellular biosynthetic process [inference]

organic acid biosynthetic process [inference]

carboxylic acid biosynthetic process [inference]

monocarboxylic acid biosynthetic process [inference]

bile acid biosynthetic process

cellular lipid metabolic process [inference]

fatty acid metabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

fatty acid oxidation [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

lipid modification [inference]

lipid oxidation [inference]

fatty acid oxidation [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

cellular lipid catabolic process [inference]

fatty acid catabolic process [inference]

fatty acid beta-oxidation [inference]

fatty acid beta-oxidation using acyl-CoA oxidase

Displaying entries **1 - 5** of 9 in total

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GO Term	Evidence Code	PMID
cytoplasm	IDA	15941950
mitochondrion	IDA	11060344 11060359 20102405 7649182
mitochondrion	IBA
mitochondrion	HTP	34800366
peroxisome	IDA	11060344 11060359 7649182

GO Hierarchy

cellular component

cellular anatomical entity [inference]

membrane [inference]

plasma membrane

membrane-enclosed lumen [inference]

organelle lumen [inference]

intracellular organelle lumen [inference]

microbody lumen [inference]

peroxisomal matrix

organelle [inference]

membrane-bounded organelle [inference]

intracellular membrane-bounded organelle

mitochondrion

microbody [inference]

peroxisome

intracellular organelle [inference]

intracellular membrane-bounded organelle

mitochondrion

microbody [inference]

peroxisome

Displaying entries **1 - 5** of 6 in total

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GO Term	Evidence Code	PMID
signaling receptor binding	IPI	11060344
alpha-methylacyl-CoA racemase activity	EXP	7649182
alpha-methylacyl-CoA racemase activity	TAS
alpha-methylacyl-CoA racemase activity	IDA	11060359 7649182
alpha-methylacyl-CoA racemase activity	IMP	10655068

GO Hierarchy

molecular function

catalytic activity [inference]

isomerase activity [inference]

racemase and epimerase activity [inference]

alpha-methylacyl-CoA racemase activity

binding [inference]

protein binding [inference]

signaling receptor binding

Gene Ontology information from NCBI Gene and UniProt.

OrthoDB (Group)

Group level

Eukaryota

Group Name

alpha-methylacyl-coa racemase

Functional Category

E: Amino acid transport and metabolism
I: Lipid transport and metabolism
Q: Secondary metabolites biosynthesis, transport and catabolism

Displaying **all 3** entries
Gene Ontology
alpha-methylacyl-CoA racemase activity
bile acid metabolic process
catalytic activity

Displaying **all 3** entries
InterPro
CoA-transferase family III domain 1 superfamily
CoA-transferase family III domain 3 superfamily
CoA-transferase family III

Human Protein Atlas

ENSG00000242110

Tissue with high expression from Human Protein Atlas. Tissues that are highly expressed are highlighted.

Data available from v23.0.proteinatlas.org. Image credit: Human Protein Atlas

KEGG BRITE Database

Orthology

K01796

Name

alpha-methylacyl-CoA racemase [EC:5.1.99.4]

References

Rhea

RHEA:12657

a (2S)-2-methylacyl-CoA

a (2R)-2-methylacyl-CoA

Enzyme

5.1.99.4

PMID

Disease

Disease Ontology

Displaying **all 3** entries
DO ID	Disease Name	Source
DOID:0060602	alpha-methylacyl-CoA racemase deficiency	Alliance of Genome Resources
DOID:0111068	congenital bile acid synthesis defect 4	Alliance of Genome Resources
DOID:11054	urinary bladder cancer	Alliance of Genome Resources

The Human Phenotype Ontology

Displaying entries **1 - 10** of 53 in total

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HPO ID	HPO Term
HP:0000007	Autosomal recessive inheritance
HP:0000135	Hypogonadism
HP:0000505	Visual impairment
HP:0000510	Rod-cone dystrophy
HP:0000518	Cataract
HP:0000580	Pigmentary retinopathy
HP:0000716	Depression
HP:0000763	Sensory neuropathy
HP:0000815	Hypergonadotropic hypogonadism
HP:0001081	Cholelithiasis

Displaying **all 3** entries
Disease ID	Disease Name
ORPHA:79095	congenital bile acid synthesis defect 4
OMIM:214950	congenital bile acid synthesis defect 4
OMIM:614307	alpha-methylacyl-CoA racemase deficiency

PubChem Disease

GHR Health Conditions

Alpha-methylacyl-CoA racemase deficiency

MedGen Diseases

OMIM Phenotypes

KEGG Diseases

Gene-Disease-Co-Occurrences-in-Literature (PubChem)

LIPID MAPS Gene / Proteome Database

LMPD ID: LMP004774
Gene Name: alpha-methylacyl-CoA racemase

Ortholog

Displaying entries **1 - 10** of 82 in total

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Species	Gene ID	Alliance of Genome Resources	Orthologous MAtrix
Mus musculus	17117		MOUSE15476
Xenopus tropicalis	23600	Xenbase:XB-GENE-1009589
Rattus norvegicus	25284		RATNO23135
Pan troglodytes	461953		PANTR35746
Bos taurus	540376		BOVIN16081
Danio rerio	553653		DANRE21037
Canis lupus familiaris	612603		CANLF15027
Macaca mulatta	698365		MACMU39741
Macaca mulatta	702339		MACMU39741
Monodelphis domestica	100015444		MONDO17536

GlyTouCan

GlyComb

GlycoPOST

UniCarb-DR

LM-GlycoRepo

All Resources

Other Information

Tools

Search Tools

Guidelines

Summary

External Links

Annotation

OrthoDB (Group)

KEGG BRITE Database

PubChem Disease

LIPID MAPS Gene / Proteome Database

International Collaboration

Acknowledgements