产品名称
GBA Rabbit Polyclonal Antibody
蛋白名称
Glucosylceramidase (EC 3.2.1.45) (Acid beta-glucosidase) (Alglucerase) (Beta-glucocerebrosidase) (D-glucosyl-N-acylsphingosine glucohydrolase) (Imiglucerase)
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=2629
Human Swissprot No.
P04062
Human Swissprot Link
https://www.uniprot.org/uniprot/P04062
Mouse Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=14466
Mouse Swissprot No.
P17439
Mouse Swissprot Link
https://www.uniprot.org/uniprotkb/P17439/entry
特异性
This antibody detects endogenous levels of GBA at Human, Mouse
运输及保存条件
-15°C to -25°C/1 year(Do not lower than -25°C)
细胞定位
Lysosome membrane ; Peripheral membrane protein ; Lumenal side . Interaction with saposin-C promotes membrane association (PubMed:10781797). Targeting to lysosomes occurs through an alternative MPR-independent mechanism via SCARB2 (PubMed:18022370). .
功能
Glucosylceramidase that catalyzes, within the lysosomal compartment, the hydrolysis of glucosylceramides/GlcCers (such as beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine) into free ceramides (such as N-acylsphing-4-enine) and glucose . Plays a central role in the degradation of complex lipids and the turnover of cellular membranes . Through the production of ceramides, participates in the PKC-activated salvage pathway of ceramide formation . Catalyzes the glucosylation of cholesterol, through a transglucosylation reaction where glucose is transferred from GlcCer to cholesterol . GlcCer containing mono-unsaturated fatty acids (such as beta-D-glucosyl-N-(9Z-octadecenoyl)-sphing-4-enine) are preferred as glucose donors for cholesterol glucosylation when compared with GlcCer containing same chain length of saturated fatty acids (such as beta-D-glucosyl-N-octadecanoyl-sphing-4-enine) . Under specific conditions, may alternatively catalyze the reverse reaction, transferring glucose from cholesteryl 3-beta-D-glucoside to ceramide (Probable). Can also hydrolyze cholesteryl 3-beta-D-glucoside producing glucose and cholesterol . Catalyzes the hydrolysis of galactosylceramides/GalCers (such as beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine), as well as the transfer of galactose between GalCers and cholesterol in vitro, but with lower activity than with GlcCers . Contrary to GlcCer and GalCer, xylosylceramide/XylCer (such as beta-D-xyosyl-(1<->1')-N-acylsphing-4-enine) is not a good substrate for hydrolysis, however it is a good xylose donor for transxylosylation activity to form cholesteryl 3-beta-D-xyloside .
