3-Hydroxy-3-methylglutaryl-CoA lyase
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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.
Quick Facts Identifiers, Aliases ...
3-hydroxy-3-methylglutaryl-CoA lyase | |||||||||||||||||||||||||||||||||||||||||||||||||||
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Aliases | HMG-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 IDs | GeneCards: | ||||||||||||||||||||||||||||||||||||||||||||||||||
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Quick Facts Hydroxymethylglutaryl-CoA lyase, Identifiers ...
Hydroxymethylglutaryl-CoA lyase | |||||||||
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Identifiers | |||||||||
EC no. | 4.1.3.4 | ||||||||
CAS no. | 9030-83-5 | ||||||||
Databases | |||||||||
IntEnz | IntEnz view | ||||||||
BRENDA | BRENDA entry | ||||||||
ExPASy | NiceZyme view | ||||||||
KEGG | KEGG entry | ||||||||
MetaCyc | metabolic pathway | ||||||||
PRIAM | profile | ||||||||
PDB structures | RCSB PDB PDBe PDBsum | ||||||||
Gene Ontology | AmiGO / QuickGO | ||||||||
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Quick Facts Identifiers, Symbol ...
3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase (hydroxymethylglutaricaciduria) | |||||||
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Identifiers | |||||||
Symbol | HMGCL | ||||||
NCBI gene | 3155 | ||||||
HGNC | 5005 | ||||||
OMIM | 246450 | ||||||
RefSeq | NM_000191 | ||||||
UniProt | P35914 | ||||||
Other data | |||||||
EC number | 4.1.3.4 | ||||||
Locus | Chr. 1 p36.1-p35 | ||||||
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