3-hydroxy-3-methylglutaryl-CoA lyase
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| Hydroxymethylglutaryl-CoA lyase | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| Identifiers | |||||||||
| EC number | 4.1.3.4 | ||||||||
| CAS number | 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 / EGO | ||||||||
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| 3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase (hydroxymethylglutaricaciduria) | |
|---|---|
| Identifiers | |
| Symbol | HMGCL |
| Entrez | 3155 |
| HUGO | 5005 |
| OMIM | 246450 |
| RefSeq | NM_000191 |
| UniProt | P35914 |
| Other data | |
| EC number | 4.1.3.4 |
| Locus | Chr. 1 p36.1-p35 |
3-hydroxy-3-methylglutaryl-CoA lyase (or HMG-CoA lyase) plays an essential role in breaking down dietary proteins and fats for energy.
Specifically, the enzyme is required for ketogenesis in the liver, and is also responsible for processing the amino acid leucine. It is found in the mitochondrion.
Pathology [edit]
Mutations in the HMGCL gene cause 3-hydroxy-3-methylglutaryl-CoA lyase deficiency.
External links [edit]
- 3-hydroxy-3-methylglutaryl-coenzyme A lyase at the US National Library of Medicine Medical Subject Headings (MeSH)
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