| Applications: |
WB |
| Note: |
STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS. |
| Short Description: |
GBA Positive Control for STJ501179 is synthetically produced from the sequence and is suitable for use in western blot applications. |
| Formulation: |
Provided as 100 uL ready-to-use, in SDS-PAGE sample buffer (Laemelli's buffer) containing Tris, pH 6.8, 1 % SDS, Glycerol and Bromophenolblue blue as tracking dye. The sample is reduced by adding 2% beta mercaptoethanol. The protein concentration is |
| Storage Instruction: |
Store at-20°C for long term storage. Avoid freeze-thaw cycles. |
| Gene Symbol: |
GBA1 |
| Gene ID: |
2629 |
| Uniprot ID: |
GBA1_HUMAN |
| Specificity: |
This is positive control is recommended for use in combination with GBA antibody STJ501179. |
| Immunogen: |
Recombinant Glucosylceramidase protein. |
| Function | 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. |
| Peptide Name | Lysosomal Acid GlucosylceramidaseLysosomal Acid GcaseAcid Beta-GlucosidaseAlgluceraseBeta-GlucocerebrosidaseBeta-GcBeta-Glucosylceramidase 1Cholesterol GlucosyltransferaseSgtaseCholesteryl-Beta-GlucosidaseD-Glucosyl-N-Acylsphingosine GlucohydrolaseGlucosylceramidase Beta 1ImigluceraseLysosomal Cholesterol GlycosyltransferaseLysosomal GalactosylceramidaseLysosomal Glycosylceramidase |
| Database Links | Reactome: R-HSA-1660662Reactome: R-HSA-390471 |
| Cellular Localisation | Lysosome MembranePeripheral Membrane ProteinLumenal SideInteraction With Saposin-C Promotes Membrane AssociationTargeting To Lysosomes Occurs Through An Alternative Mpr-Independent Mechanism Via Scarb2 |
| Alternative Peptide Names | Lysosomal Acid Glucosylceramidase proteinLysosomal Acid Gcase proteinAcid Beta-Glucosidase proteinAlglucerase proteinBeta-Glucocerebrosidase proteinBeta-Gc proteinBeta-Glucosylceramidase 1 proteinCholesterol Glucosyltransferase proteinSgtase proteinCholesteryl-Beta-Glucosidase proteinD-Glucosyl-N-Acylsphingosine Glucohydrolase proteinGlucosylceramidase Beta 1 proteinImiglucerase proteinLysosomal Cholesterol Glycosyltransferase proteinLysosomal Galactosylceramidase proteinLysosomal Glycosylceramidase proteinGBA1 proteinGBA proteinGC proteinGLUC protein |
Information sourced from Uniprot.org
12 months for antibodies. 6 months for ELISA Kits. Please see website T&Cs for further guidance
