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HMGCL Antibody (Center) Blocking Peptide

Synthetic peptide

     
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Product Information
Primary Accession P35914
Clone Names 90121166
Additional Information
Gene ID 3155
Other Names Hydroxymethylglutaryl-CoA lyase, mitochondrial, HL, HMG-CoA lyase, 3-hydroxy-3-methylglutarate-CoA lyase, HMGCL
Format Peptides are lyophilized in a solid powder format. Peptides can be reconstituted in solution using the appropriate buffer as needed.
StorageMaintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.
PrecautionsThis product is for research use only. Not for use in diagnostic or therapeutic procedures.
Protein Information
Name HMGCL
Function Mitochondrial 3-hydroxymethyl-3-methylglutaryl-CoA lyase that catalyzes a cation-dependent cleavage of (S)-3-hydroxy-3- methylglutaryl-CoA into acetyl-CoA and acetoacetate, a key step in ketogenesis. 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 {ECO:0000250|UniProtKB:P38060}. Peroxisome {ECO:0000250|UniProtKB:P38060}. Note=Unprocessed form is peroxisomal {ECO:0000250|UniProtKB:P38060}
Tissue Location Highest expression in liver. Expressed in pancreas, kidney, 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%).
Research Areas
Citations (0)
citation

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Background

HMGCL 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].

References

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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$ 277.78
Cat# BP16221c
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