UniProt:Q13825 AUH

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.
crossReference
databaseName UniProt
dbId 85696
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
displayName UniProt:Q13825 AUH
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:9948485] Weiser, Joel, 2025-05-21
moleculeType Protein
name
  • AUH
otherIdentifier
  • 11745450_a_at
  • 11749988_x_at
  • 17095686
  • 205052_PM_at
  • 205052_at
  • 3214373
  • 3214374
  • 3214375
  • 3214378
  • 3214380
  • 3214385
  • 3214386
  • 3214400
  • 3214401
  • 3214405
  • 3214406
  • 3214412
  • 3214415
  • 3214416
  • 3214417
  • 3214420
  • 3214421
  • 3214422
  • 3214423
  • 3214424
  • 37616_at
  • 549
  • 8162264
  • A_23_P20852
  • GE60058
  • GO:0003723
  • GO:0003730
  • GO:0003824
  • GO:0004300
  • GO:0004490
  • GO:0005739
  • GO:0005759
  • GO:0006520
  • GO:0006552
  • GO:0006629
  • GO:0006635
  • GO:0009083
  • GO:0016829
  • GO:0050011
  • HMNXSV003029040
  • HMNXSV003045621
  • ILMN_2231911
  • PH_hs_0004678
  • TC09001324.hg
  • X79888_at
  • g4502326_3p_at
physicalEntity
referenceDatabase [ReferenceDatabase:2] UniProt
referenceGene
referenceTranscript
schemaClass ReferenceGeneProduct
secondaryIdentifier
  • AUHM_HUMAN
  • B1ALV7
  • B1ALV8
  • Q8WUE4
sequenceLength 339
species [Species:48887] Homo sapiens
stId uniprot:Q13825
url http://purl.uniprot.org/uniprot/Q13825
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