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GC Antibody(Center) (Ascites)

Mouse Monoclonal Antibody (Mab)

     
  • 1 - GC Antibody(Center) (Ascites) AM2180a
    GC Antibody(Center) (Cat. #AM2180a) western blot analysis in MCF-7 cell line lysates (35μg/lane).This demonstrates the GC antibody detected the GC protein (arrow).
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Product info
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
WB, E
Primary Accession P04062
Other Accession Q70KH2, Q2KHZ8, NP_000148.2
Reactivity Human
Predicted Bovine, Pig
Host Mouse
Clonality Monoclonal
Isotype IgM
Clone Names 660CT8.6.6.2
Calculated MW 59716 Da
Additional info
Gene ID 2629
Other Names Glucosylceramidase, Acid beta-glucosidase, Alglucerase, Beta-glucocerebrosidase, Beta-GC, D-glucosyl-N-acylsphingosine glucohydrolase, Imiglucerase, GBA, GC, GLUC
Target/Specificity This GC antibody is generated from mice immunized with a KLH conjugated synthetic peptide between 337-365 amino acids from the Central region of human GC.
Dilution WB~~1:100~1600
Format Mouse monoclonal antibody supplied in crude ascites with 0.09% (W/V) sodium azide.
StorageMaintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.
PrecautionsGC Antibody(Center) (Ascites) is for research use only and not for use in diagnostic or therapeutic procedures.
Protein Information
Name GBA (HGNC:4177)
Synonyms GC, GLUC
Function Glucosylceramidase that catalyzes, within the lysosomal compartment, the hydrolysis of glucosylceramide/GlcCer into free ceramide and glucose (PubMed:9201993, PubMed:24211208). Thereby, plays a central role in the degradation of complex lipids and the turnover of cellular membranes (PubMed:27378698). Through the production of ceramides, participates to the PKC-activated salvage pathway of ceramide formation (PubMed:19279011). Also plays a role in cholesterol metabolism (PubMed:24211208, PubMed:26724485). May either catalyze the glucosylation of cholesterol, through a transglucosylation reaction that transfers glucose from glucosylceramide to cholesterol (PubMed:24211208, PubMed:26724485). The short chain saturated C8:0-GlcCer and the mono-unsaturated C18:0-GlcCer being the most effective glucose donors for that transglucosylation reaction (PubMed:24211208). Under specific conditions, may alternatively catalyze the reverse reaction, transferring glucose from cholesteryl-beta-D-glucoside to ceramide (PubMed:26724485). Finally, may also hydrolyze cholesteryl-beta-D-glucoside to produce D-glucose and cholesterol (PubMed:24211208, PubMed:26724485).
Cellular Location Lysosome membrane; Peripheral membrane protein; Lumenal side. Note=Interaction with saposin-C promotes membrane association (PubMed:10781797). Targeting to lysosomes occurs through an alternative MPR-independent mechanism via SCARB2 (PubMed:18022370).
Research Areas

BACKGROUND

This gene encodes a lysosomal membrane protein that cleaves the beta-glucosidic linkage of glycosylceramide, an intermediate in glycolipid metabolism. Mutations in this gene cause Gaucher disease, a lysosomal storage disease characterized by an accumulation of glucocerebrosides. A related pseudogene is approximately 12 kb downstream of this gene on chromosome 1. Alternative splicing results in multiple transcript variants.

REFERENCES

Dos Santos, A.V., et al. Neurosci. Lett. 485(2):121-124(2010)
Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010)
Jeong, S.Y., et al. Blood Cells Mol. Dis. (2010) In press :
Hu, F.Y., et al. Eur. J. Neurol. (2010) In press :
Velayati, A., et al. Curr Neurol Neurosci Rep 10(3):190-198(2010)

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