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- Table of Contents
Facts about Methylglutaconyl-CoA hydratase, mitochondrial.
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).
Human | |
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Gene Name: | AUH |
Uniprot: | Q13825 |
Entrez: | 549 |
Belongs to: |
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enoyl-CoA hydratase/isomerase family |
3-methylglutaconyl-CoA hydratase; AU RNA binding protein/enoyl-CoA hydratase; AU RNA binding protein/enoyl-Coenzyme A hydratase; AU RNA-binding protein/enoyl-Coenzyme A hydratase; AU-binding protein/enoyl-CoA hydratase; AU-specific RNA-binding enoyl-CoA hydratase; EC 4.2.1.18; methylglutaconyl-CoA hydratase, mitochondrial
Mass (kDA):
35.609 kDA
Human | |
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Location: | 9q22.31 |
Sequence: | 9; NC_000009.12 (91213815..91361969, complement) |
Mitochondrion.
PMID: 7892223 by Nakagawa J., et al. AUH, a gene encoding an AU-specific RNA binding protein with intrinsic enoyl-CoA hydratase activity.
PMID: 12434311 by Ijlst L., et al. 3-methylglutaconic aciduria type I is caused by mutations in AUH.