Jump to Products:
• Monoclonal Antibodies >

Hydroxymethylglutaryl-CoA lyase (HMGCL)

UniProt Number: P35914
Alternate Names: HMG-CoA lyase, HL, 3-hydroxy-3-methylglutarate-CoA lyase
Structure and Function: HMGCL is a mitochondrial enzyme that is involved in the catabolism of branched amino acids such as leucine. HMGCL is also involved in the production of ketones during the breakdown of fats by converting β-hydroxy-β-methylglutaryl-CoA to acetyl-CoA and acetoacetate.
Disease Associations: Defects in HMGCL are the cause of 3-hydroxy-3-methylglutaryl-CoA lyase deficiency (HMGCLD) [MIM:246450] (also known as hydroxymethylglutaricaciduria or HL deficiency), an autosomal recessive disease affecting ketogenesis and L-leucine catabolism.

Monoclonal Antibodies
Cat. No. Name Reactivity Apps. Amount
MS725 HMGCL antibody human ICC, IP, ICE 100 µg

Browse Products By:
Product Search:

Related Pathways:

Acetyl-CoA Metabolism: Key Nodes


Subscribe to MitoNews
MitoNews is a free periodic annotated review of important publications in mitochondria and metabolic research.

MitoNews Latest Issue
Volume 11 Issue 01 (February 2015)

MitoNews Archives

Sales & Customer Support:

[email protected]

© 2004-2022 MitoSciences Inc, an Abcam company. All rights reserved.