Comparison

Recombinant Human Hydroxymethylglutaryl-CoA lyase, mitochondrial(HMGCL)

Manufacturer Cusabio
Category
Type Proteins
Specific against other
Amount 10ug
Host E.coli
Item no. CSB-EP010563HU-10
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
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%).
Amount: 10ug
Available: In stock
Listprice: €175.45
Price: €175.45
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