AAH10570.1
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
20,222 Da
NCBI Official Full Name
3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase
NCBI Official Synonym Full Names
3-hydroxymethyl-3-methylglutaryl-CoA lyase
NCBI Official Synonym Symbols
NCBI Protein Information
hydroxymethylglutaryl-CoA lyase, mitochondrial
UniProt Protein Name
Hydroxymethylglutaryl-CoA lyase, mitochondrial
UniProt Synonym Protein Names
3-hydroxy-3-methylglutarate-CoA lyase
UniProt Synonym Gene Names
NCBI Summary for HMGCL
The protein encoded by this gene belongs to the HMG-CoA lyase family. It is a mitochondrial enzyme that catalyzes the final step of leucine degradation and plays a key role in ketone body formation. Mutations in this gene are associated with HMG-CoA lyase deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2009]
UniProt Comments for HMGCL
Key enzyme in ketogenesis (ketone body formation). Terminal step in leucine catabolism. Ketone bodies (beta-hydroxybutyrate, acetoacetate and acetone) are essential as an alternative source of energy to glucose, as lipid precursors and as regulators of metabolism.
Research Articles on HMGCL
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Products associated with HMGCL antibody pair kit
Pathways associated with HMGCL antibody pair kit
Diseases associated with HMGCL antibody pair kit
Organs/Tissues associated with HMGCL antibody pair kit
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