Anti-Glucosylceramidase beta antibody (40-250) (STJ11102788)
SPECIFICATIONS
ClonalityPolyclonal
HostRabbit
ConjugationUnconjugated
IsotypeIgG
General Information
| Short Description | Rabbit polyclonal Glucosylceramidase beta (GBA) (40-250) antibody for use in WB and ELISA in human and rat samples. Datasheet included with dilution recommendations, and related reagents. |
| Applications | WB/ELISA |
| Host | Rabbit |
| Reactivity | Human/Rat |
| Note | STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS. |
Product Properties
| Clonality | Polyclonal |
| Isotype | IgG |
| Conjugation | Unconjugated |
| Concentration | Lot specific |
| Purification | Affinity purification |
| Dilution Range | WB:1:500-1:2000ELISA:Recommended starting concentration is 1 Mu g/mL. Please optimize the concentration based on your specific assay requirements. |
| Formulation | PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. |
| Storage Instruction | Store at-20ยฐC for up to 1 year from the date of receipt, and avoid repeat freeze-thaw cycles. |
Target Information
| Gene Symbol | GBA1 |
| Gene ID | 2629 |
| Uniprot ID | GBA1_HUMAN |
| Immunogen Region | 40-250 |
| Immunogen Sequence | ARPCIPKSFGYSSVVCVCNA TYCDSFDPPTFPALGTFSRY ESTRSGRRMELSMGPIQANH TGTGLLLTLQPEQKFQKVKG FGGAMTDAAALNILALSPPA QNLLLKSYFSEEGIGYNIIR VPMASCDFSIRTYTYADTPD DFQLHNFSLPEEDTKLKIPL IHRALQLAQRPVSLLASPWT SPTWLKTNGAVNGKGSLKGQ PGDIYHQTWAR |
| Specificity | Recombinant fusion protein containing a sequence corresponding to amino acids 40-250 of human Glucosylceramidase beta (Glucosylceramidase beta (GBA) ) (NP_000148.2). |
Additional Info
| 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. |
| Protein 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-390471Reactome: R-HSA-9840310 |
| 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 Antibody Names | Anti-Lysosomal Acid Glucosylceramidase antibodyAnti-Lysosomal Acid Gcase antibodyAnti-Acid Beta-Glucosidase antibodyAnti-Alglucerase antibodyAnti-Beta-Glucocerebrosidase antibodyAnti-Beta-Gc antibodyAnti-Beta-Glucosylceramidase 1 antibodyAnti-Cholesterol Glucosyltransferase antibodyAnti-Sgtase antibodyAnti-Cholesteryl-Beta-Glucosidase antibodyAnti-D-Glucosyl-N-Acylsphingosine Glucohydrolase antibodyAnti-Glucosylceramidase Beta 1 antibodyAnti-Imiglucerase antibodyAnti-Lysosomal Cholesterol Glycosyltransferase antibodyAnti-Lysosomal Galactosylceramidase antibodyAnti-Lysosomal Glycosylceramidase antibodyAnti-GBA1 antibodyAnti-GBA antibodyAnti-GC antibodyAnti-GLUC antibody |
Information sourced from Uniprot.org