Abcam, ab97293, Anti-HMGCL antibody

Size: 50µL
Rabbit Polyclonal HMGCL antibody. Suitable for WB, ICC/IF and reacts with Mouse, Rat, Human samples. Cited in 2 publications. Immunogen corresponding to Recombinant Fragment Protein within Human HMGCL aa 1-200.
Key facts
Host species:Rabbit,
Clonality:Polyclonal,
Isotype:IgG,
Carrier free:No,
Reacts with:Mouse, Rat, Human,
Applications:ICC/IF, WBSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:Recombinant Fragment Protein within Human HMGCL aa 1-200. The exact immunogen used to generate this antibody is proprietary information.P35914

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Immunogen, Purification notes-Purified by antigen affinity chromatography., Storage buffer-pH: 7Preservative: 0.01% Thimerosal (merthiolate)Constituents: 10% Glycerol (glycerin, glycerine), 1.21% Tris, 0.75% Glycine, Shipped at conditions-Blue Ice, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze / thaw cycle

Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
HMGCL or 3-hydroxymethyl-3-methylglutaryl-CoA lyase plays an important role in ketogenesis and leucine catabolism. This enzyme catalyzes the cleavage of HMG-CoA to acetoacetate and acetyl-CoA. It has a molecular mass of approximately 33 kDa. HMGCL expresses mainly in the liver kidney and brain where ketogenesis is active signifying its essential role in energy metabolism in fasting conditions.
Biological function summary
This enzyme contributes to the production of ketone bodies important components during periods of low glucose availability. HMGCL does not function as part of a multi-protein complex but its action underpins the metabolic switch from glucose to fatty acids and ketone body usage. Proper functioning of this enzyme helps maintain energy balance especially during fasting and extended exercise.
Pathways
HMGCL plays a significant role in the ketogenic pathway linking fatty acid oxidation to ketone body production. It also intersects with leucine degradation pathways reflecting its dual substrate specificity. HMGCL shares links with proteins involved in lipid metabolism such as HMG-CoA synthase which provides the substrate for its enzymatic activity.
Defects in HMGCL associate with HMG-CoA lyase deficiency a metabolic disorder hallmarked by hypoketotic hypoglycemia and metabolic acidosis. This condition highlights the enzyme's role in ketogenesis. Additionally its impaired function links to potential exacerbation of seizures due to energy metabolism failure. The enzyme's activity correlates with enzymes like carnitine palmitoyltransferase I which also impact energy metabolism through ketogenesis.