3-Hydroxy-3-methylglutaryl-CoA lyase

3-hydroxy-3-methylglutaryl-CoA lyase
Identifiers
AliasesHMG-CoA_lyasehydroxymethylglutaryl-CoA lyaseHMG-CoA lyase3-hydroxy-3-methylglutaryl coenzyme A lyase(S)-3-hydroxy-3-methylglutaryl-CoA acetoacetate-lyase (acetyl-CoA-forming)(S)-3-hydroxy-3-methylglutaryl-CoA acetoacetate-lyase3-hydroxy-3-methylglutarate-CoA lyasehydroxymethylglutaryl coenzyme A lyase
External IDsGeneCards: ; OMA:- orthologs
Orthologs
SpeciesHumanMouse
Entrez

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Ensembl

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UniProt

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RefSeq (mRNA)

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RefSeq (protein)

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Location (UCSC)n/an/a
PubMed searchn/an/a
Wikidata
View/Edit Human
Hydroxymethylglutaryl-CoA lyase
HMG-CoA lyase dimer, Human
Identifiers
EC no.4.1.3.4
CAS no.9030-83-5
Databases
IntEnzIntEnz view
BRENDABRENDA entry
ExPASyNiceZyme view
KEGGKEGG entry
MetaCycmetabolic pathway
PRIAMprofile
PDB structuresRCSB PDB PDBe PDBsum
Gene OntologyAmiGO / QuickGO
Search
PMCarticles
PubMedarticles
NCBIproteins
3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase (hydroxymethylglutaricaciduria)
Identifiers
SymbolHMGCL
NCBI gene3155
HGNC5005
OMIM246450
RefSeqNM_000191
UniProtP35914
Other data
EC number4.1.3.4
LocusChr. 1 p36.1-p35
Search for
StructuresSwiss-model
DomainsInterPro

3-Hydroxy-3-methylglutaryl-CoA lyase (or HMG-CoA lyase) is an enzyme (EC 4.1.3.4 that in human is encoded by the HMGCL gene located on chromosome 1. It is a key enzyme in ketogenesis (ketone body formation). It is a ketogenic enzyme in the liver that catalyzes the formation of acetoacetate from HMG-CoA within the mitochondria. It also plays a prominent role in the catabolism of the amino acid leucine.

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