3-Hydroxy-3-methylglutaryl-CoA lyase
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.
| 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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| Hydroxymethylglutaryl-CoA lyase | |||||||||
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HMG-CoA lyase dimer, Human | |||||||||
| 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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| 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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