|Other Names||Hydroxymethylglutaryl-CoA lyase, mitochondrial, HL, HMG-CoA lyase, 3-hydroxy-3-methylglutarate-CoA lyase, HMGCL|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||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.|
|Cellular Location||Mitochondrion matrix. Peroxisome. Note=Unprocessed form is peroxisomal|
|Tissue Location||Highest expression in liver. Expressed in pancreas, kidey, intestine, testis, fibroblasts and lymphoblasts Very low expression in brain and skeletal muscle. The relative expression of isoform 2 (at mRNA level) is highest in heart (30%), skeletal muscle (22%), and brain (14%)|
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Provided below are standard protocols that you may find useful for product applications.
The protein encoded by this gene belongs to the HMG-CoAlyase family. It is a mitochondrial enzyme that catalyzes the finalstep of leucine degradation and plays a key role in ketone bodyformation. Mutations in this gene are associated with HMG-CoA lyasedeficiency. Alternatively spliced transcript variants encodingdifferent isoforms have been found for this gene. [provided byRefSeq].
Fu, Z., et al. J. Biol. Chem. 285(34):26341-26349(2010)Pierron, S., et al. Arch Pediatr 17(1):10-13(2010)Menao, S., et al. Hum. Mutat. 30 (3), E520-E529 (2009) :Lin, W.D., et al. Clin. Chim. Acta 401 (1-2), 33-36 (2009) :Carrasco, P., et al. Mol. Genet. Metab. 91(2):120-127(2007)
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