Recombinant Human Hydroxymethylglutaryl-CoA lyase, mitochondrial(HMGCL)

Code CSB-EP010563HU
Product Type Recombinant Protein
Size US$1726
Uniprot No. P35914
Relevance 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.
Image
  • (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.

Storage Buffer Tris-based buffer,50% glycerol
Alias 3-hydroxy-3-methylglutarate-CoA lyase
Species Homo sapiens (Human)
Purity Greater than 90% as determined by SDS-PAGE.
Sequence TLPKRVKIVEVGPRDGLQNEKNIVSTPVKIKLIDMLSEAGLSVIETTSFVSPKWVPQMGDHTEVLKGIQKFPGINYPVLTPNLKGFEAAVAAGAKEVVIFGAASELFTKKNINCSIEESFQRFDAILKAAQSANISVRGYVSCALGCPYEGKISPAKVAEVTKKFYSMGCYEISLGDTIGVGTPGIMKDMLSAVMQEVPLAALAVHCHDTYGQALANTLMALQMGVSVVDSSVAGLGGCPYAQGASGNLATEDLVYMLEGLGIHTGVNLQKLLEAGNFICQALNRKTSSKVAQATCKL
Research Area Signal Transduction
Source E.coli
Gene Names HMGCL
Protein Names Recommended name: Hydroxymethylglutaryl-CoA lyase, mitochondrial Short name= HL Short name= HMG-CoA lyase EC= 4.1.3.4 Alternative name(s): 3-hydroxy-3-methylglutarate-CoA lyase
Expression Region 1-325aa
Tag Info N-terminal GST-tagged
Mol. Weight 58.5kDa
Protein Description Full Length
Storage The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself. Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
Notes Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
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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.
Involvement in disease 3-hydroxy-3-methylglutaryl-CoA lyase deficiency (HMGCLD)
Subcellular Location Mitochondrion matrix, Peroxisome
Protein Families HMG-CoA lyase family
Tissue Specificity 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
Database Links

HGNC: 5005

OMIM: 246450

KEGG: hsa:3155

STRING: 9606.ENSP00000363614

UniGene: Hs.533444

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