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Details on Person UniProt:Q13825 AUH

Class:Id	ReferenceGeneProduct:85696
_chainChangeLog	transit peptide:1-67 added on Fri February 6 2015;chain:68-339 added on Fri February 6 2015
_displayName	UniProt:Q13825 AUH
_timestamp	2025-05-21 21:04:03
chain	transit peptide:1-67 chain:68-339
checksum	E04FEB95933FB30B
comment	FUNCTION Catalyzes the fifth step in the leucine degradation pathway, the reversible hydration of 3-methylglutaconyl-CoA (3-MG-CoA) to 3-hydroxy-3-methylglutaryl-CoA (HMG-CoA) (PubMed:11738050, PubMed:12434311, PubMed:12655555, PubMed:16640564). Can catalyze the reverse reaction but at a much lower rate in vitro (PubMed:16640564). HMG-CoA is then quickly degraded by another enzyme (such as HMG-CoA lyase) to give acetyl-CoA and acetoacetate (PubMed:16640564). Uses other substrates such as (2E)-glutaconyl-CoA efficiently in vitro, and to a lesser extent 3-methylcrotonyl-CoA (3-methyl-(2E)-butenoyl-CoA), crotonyl-CoA ((2E)-butenoyl-CoA) and 3-hydroxybutanoyl-CoA (the missing carboxylate reduces affinity to the active site) (PubMed:16640564). Originally it was identified as an RNA-binding protein as it binds to AU-rich elements (AREs) in vitro (PubMed:7892223). AREs direct rapid RNA degradation and mRNA deadenylation (PubMed:7892223). Might have itaconyl-CoA hydratase activity, converting itaconyl-CoA into citramalyl-CoA in the C5-dicarboxylate catabolism pathway (PubMed:29056341). The C5-dicarboxylate catabolism pathway is required to detoxify itaconate, an antimicrobial metabolite and immunomodulator produced by macrophages during certain infections, that can act as a vitamin B12-poisoning metabolite (PubMed:29056341).CATALYTIC ACTIVITY (3S)-3-hydroxy-3-methylglutaryl-CoA = 3-methyl-(2E)-glutaconyl-CoA + H2OCATALYTIC ACTIVITY (3S)-citramalyl-CoA = itaconyl-CoA + H2OCATALYTIC ACTIVITY 3-hydroxyisovaleryl-CoA = 3-methylbut-2-enoyl-CoA + H2OCATALYTIC ACTIVITY (S)-3-hydroxyglutaryl-CoA = (2E)-glutaconyl-CoA + H2OBIOPHYSICOCHEMICAL PROPERTIES Amino-acid degradation; L-leucine degradation; (S)-3-hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA: step 3/3.SUBUNIT Homohexamer.SUBCELLULAR LOCATION The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the enoyl-CoA hydratase/isomerase family.
description	recommendedName: fullName evidence="8"Methylglutaconyl-CoA hydratase, mitochondrial shortName evidence="8"3-MG-CoA hydratase ecNumber evidence="3 5"4.2.1.18 alternativeName: AU-specific RNA-binding enoyl-CoA hydratase shortName: AU-binding protein/enoyl-CoA hydratase alternativeName: fullName evidence="9"Itaconyl-CoA hydratase ecNumber evidence="9"4.2.1.56
geneName	AUH
identifier	Q13825
isSequenceChanged	FALSE
keyword	3D-structure Acetylation Alternative splicing Branched-chain amino acid catabolism Direct protein sequencing Disease variant Lyase Mitochondrion Proteomics identification Reference proteome RNA-binding Transit peptide
modified	[InstanceEdit:9836292] Weiser, Joel, 2023-05-25 [InstanceEdit:9852000] Weiser, Joel, 2023-11-03 [InstanceEdit:9917590] Weiser, Joel, 2024-08-09 [InstanceEdit:9926675] Weiser, Joel, 2024-11-03 [InstanceEdit:9939033] Weiser, Joel, 2025-02-21 [InstanceEdit:9948485] Weiser, Joel, 2025-05-21
name	AUH
referenceDatabase	[ReferenceDatabase:2] UniProt
referenceGene	[ReferenceDNASequence:9004553] ENSEMBL:ENSG00000148090 AUH [Homo sapiens]
secondaryIdentifier	AUHM_HUMAN B1ALV7 B1ALV8 Q8WUE4
sequenceLength	339
species	[Species:48887] Homo sapiens
(isoformParent)	[ReferenceIsoform:144417] UniProt:Q13825-2 AUH [Homo sapiens] [ReferenceIsoform:405663] UniProt:Q13825-1 AUH [Homo sapiens]
(referenceEntity)	[EntityWithAccessionedSequence:70775] AUH [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9914312] G217D AUH [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9914314] R331* AUH [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9914316] A240V AUH [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9914323] R197* AUH [mitochondrial matrix] [Homo sapiens]
(referenceSequence)	[NonsenseMutation:9914313] Nonsense mutation at L-lysine 331 [NonsenseMutation:9914317] Nonsense mutation at L-arginine 197 [ReplacedResidue:9914319] glycine 217 replaced with L-aspartic acid [ReplacedResidue:9914322] L-alanine 240 replaced with L-valine
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No pathways have been reviewed or authored by UniProt:Q13825 AUH (85696)