InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: AUH

Summary

InnateDB Protein

IDBP-75674.7

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

AUH

Protein Name

AU RNA binding protein/enoyl-CoA hydratase

Synonyms

Species

Homo sapiens

Ensembl Protein

ENSP00000307334

InnateDB Gene

IDBG-75670 (AUH)

Protein Structure

UniProt Annotation

Function

Catalyzes the conversion of 3-methylglutaconyl-CoA to 3- hydroxy-3-methylglutaryl-CoA. Has very low enoyl-CoA hydratase activity. Was originally identified as RNA-binding protein that binds in vitro to clustered 5'-AUUUA-3' motifs. {ECO:0000269PubMed:11738050, ECO:0000269PubMed:12434311, ECO:0000269PubMed:12655555, ECO:0000269PubMed:7892223}.

Subcellular Localization

Mitochondrion {ECO:0000250}.

Disease Associations

3-methylglutaconic aciduria 1 (MGA1) [MIM:250950]: An inborn error of leucine metabolism. It leads to an autosomal recessive syndrome with variable clinical phenotype, ranging from delayed speech development to severe psychomotor retardation, coma, failure to thrive, metabolic acidosis and dystonia. MGA1 can be distinguished from other forms of MGA by the pattern of metabolite excretion: 3-methylglutaconic acid levels are higher than those detected in other forms, whereas methylglutaric acid levels are usually only slightly elevated and there is a high level of 3-hydroxyisovaleric acid excretion (not present in other MGA forms). {ECO:0000269PubMed:12655555}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 3 experimentally validated interaction(s) in this database.

Experimentally validated
Total	3 [view]
Protein-Protein	3 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0003730	mRNA 3'-UTR binding
GO:0003824	catalytic activity
GO:0004300	enoyl-CoA hydratase activity
GO:0004490	methylglutaconyl-CoA hydratase activity

Biological Process

GO:0006552	leucine catabolic process
GO:0008152	metabolic process
GO:0009083	branched-chain amino acid catabolic process
GO:0034641	cellular nitrogen compound metabolic process
GO:0044281	small molecule metabolic process