Tbio AUH Methylglutaconyl-CoA hydratase, mitochondrial

Protein Summary

Description

Catalyzes the conversion of 3-methylglutaconyl-CoA to 3-hydroxy-3-methylglutaryl-CoA (PubMed:11738050, PubMed:12434311, PubMed:12655555). Also has itaconyl-CoA hydratase activity by converting itaconyl-CoA into citramalyl-CoA in the C5-dicarboxylate catabolism pathway (PubMed:29056341). The C5-dicarboxylate catabolism pathway is required to detoxify itaconate, a vitamin B12-poisoning metabolite (PubMed:29056341). Has very low enoyl-CoA hydratase activity (PubMed:7892223). Was originally identified as RNA-binding protein that binds in vitro to clustered 5'-AUUUA-3' motifs (PubMed:7892223). This gene encodes bifunctional mitochondrial protein that has both RNA-binding and hydratase activities. The encoded protein is a methylglutaconyl-CoA hydratase that catalyzes the hydration of 3-methylglutaconyl-CoA to 3-hydroxy-3-methyl-glutaryl-CoA, a critical step in the leucine degradation pathway. This protein also binds AU-rich elements (AREs) found in the 3' UTRs of rapidly decaying mRNAs including c-fos, c-myc and granulocyte/ macrophage colony stimulating factor. ARE elements are involved in directing RNA to rapid degradation and deadenylation. This protein is localizes to the mitochondrial matrix and the inner mitochondrial membrane and may be involved in mitochondrial protein synthesis. Mutations in this gene are the cause of 3-methylglutaconic aciduria, type I. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Sep 2015]

Uniprot Accession IDs

• Q13825
• B1ALV7
• B1ALV8
• Q8WUE4

Gene Name

• AUH

Ensembl ID

• ENST00000303617
• ENSP00000307334
• ENSG00000148090
• ENST00000375731
• ENSP00000364883

Knowledge Table

Most Knowledge About	Knowledge Value (0 to 1 scale)
transcription factor perturbation	0.96
disease perturbation	0.93
virus perturbation	0.88
kinase perturbation	0.84
cell type or tissue	0.79

Protein Classes

PANTHER Classes

Protein / Transferase / Acyltransferase / Methylglutaconyl-coa Hydratase, Mitochondrial

Protein / Transferase / Acetyltransferase / Methylglutaconyl-coa Hydratase, Mitochondrial

Protein / Oxidoreductase / Dehydrogenase / Methylglutaconyl-coa Hydratase, Mitochondrial

Protein / Lyase / Hydratase / Methylglutaconyl-coa Hydratase, Mitochondrial

Protein / Ligase / Methylglutaconyl-coa Hydratase, Mitochondrial

Protein / Isomerase / Epimerase/racemase / Methylglutaconyl-coa Hydratase, Mitochondrial

DTO Classes

Protein / Enzyme / Isomerase / Epimerase/racemase / Methylglutaconyl-coa Hydratase, Mitochondrial

IDG Development Level Summary

Tdark

These are targets about which virtually nothing is known. They do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 209.13   (req: < 5)

Gene RIFs: 8   (req: <= 3)

Antibodies: 134   (req: <= 50)

Tbio

These targets do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 209.13   (req: >= 5)

Gene RIFs: 8   (req: > 3)

Antibodies: 134   (req: > 50)

- OR - satisfy the following criterion:

Gene Ontology Terms: 7

Tchem

Target has at least one ChEMBL compound with an activity cutoff of < 30 nM - AND - satisfies the preceding conditions

Active Ligand: 0

Tclin

Target has at least one approved drug - AND - satisfies the preceding conditions

Active Drug: 0