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Recombinant Human Hydroxymethylglutaryl-CoA lyase, mitochondrial(HMGCL)

Recombinant Human Hydroxymethylglutaryl-CoA lyase, mitochondrial(HMGCL)

Manufacturer	Cusabio
Category	Peptides & Proteins Recombinant Proteins
Type	Proteins Recombinant
Specific against	other
Format	Liquid or Lyophilized powder
Amount	10ug
Host	E.coli
Item no.	CSB-EP010563HU-10
Conjugate/Tag	GST
eClass 6.1	34160400
eClass 9.0	42020190
Available
Research Topic	Signal Transduction
Uniprot ID	P35914
Gene Names	HMGCL
Organism	Homo sapiens (Human)
AA Sequence	TLPKRVKIVEVGPRDGLQNEKNIVSTPVKIKLIDM LSEAGLSVIETTSFVSPKWVPQMGDHTEVLKGIQK FPGINYPVLTPNLKGFEAAVAAGAKEVVIFGAASE LFTKKNINCSIEESFQRFDAILKAAQSANISVRGY VSCALGCPYEGKISPAKVAEVTKKFYSMGCYEISL GDTIGVGTPGIMKDMLSAVMQEVPLAALAVHCHDT YGQALANTLMALQMGVSVVDSSVAGLGGCPYAQGA SGNLATEDLVYMLEGLGIHTGVNLQKLLEAGNFIC QALNRKTSSKVAQATCKL
Expression Region	1-325aa
Sequence Info	Full Length
Source	E.coli
Tag Info	N-terminal GST-tagged
MW	58.5 kDa
Alternative Name(s)	3-hydroxy-3-methylglutarate-CoA lyase
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.
Reference	"3-hydroxy-3-methylglutaryl coenzyme A lyase (HL). Cloning of human and chicken liver HL cDNAs and characterization of a mutation causing human HL deficiency." Mitchell G.A., Robert M.-F., Hruz P.W., Wang S., Fontaine G., Behnke C.E., Mende-Mueller L.M., Schappert K., Lee C., Gibson K.M., Miziorko H.M. J. Biol. Chem. 268:4376-4381(1993)
Purity	Greater than 90% as determined by SDS-PAGE.
Storage Buffer	Tris-based buffer, 50% glycerol
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 -20C/-80C. The shelf life of lyophilized form is 12 months at -20C/-80C.
Notes	Repeated freezing and thawing is not recommended. Store working aliquots at 4C for up to one week.
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 (22%), and brain (14%).
Tag Information	N-terminal GST-tagged

Note: The presented information and documents (Manual, Product Datasheet, Safety Datasheet and Certificate of Analysis) correspond to our latest update and should serve for orientational purpose only. We do not guarantee the topicality. We would kindly ask you to make a request for specific requirements, if necessary.

All products are intended for research use only (RUO). Not for human, veterinary or therapeutic use.

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