|Application ||WB, E|
|Other Accession||Q70KH2, Q2KHZ8, NP_000148.2|
|Calculated MW||59716 Da|
|Antigen Region||337-365 aa|
|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.|
|Format||Mouse monoclonal antibody supplied in crude ascites with 0.09% (W/V) sodium azide.|
|Storage||Maintain 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.|
|Precautions||GC Antibody(Center) (Ascites) is for research use only and not for use in diagnostic or therapeutic procedures.|
|Cellular Location||Lysosome membrane; Peripheral membrane protein; Lumenal side. Note=Interaction with saposin-C promotes membrane association. Targeting to lysosomes occurs through an alternative MPR-independent mechanism via SCARB2|
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Provided below are standard protocols that you may find useful for product applications.
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.
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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