Hersteller |
Cusabio
|
Kategorie |
|
Typ |
Proteins Recombinant |
Specific against |
other |
Format |
Liquid or Lyophilized powder |
Menge |
10ug |
Host |
E.coli |
ArtNr |
CSB-EP010563HU-10 |
Konjugat/Tag |
GST |
eClass 6.1 |
34160400 |
eClass 9.0 |
42020190 |
Lieferbar |
|
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 |
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