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127196 Mouse Anti-GBA (Glucosylceramidase, Acid beta-glucosidase, Alglucerase, Beta-glucocerebrosidase, D-glucosyl-N-acylsphingosine Glucohydrolase, Imiglucerase, GC, GLUC)

Specifications
References
Clone Type
Monoclonal
Host
Mouse
Source
Human
Isotype
IgG2a,k
Clone Number
2e2
Grade
Affinity Purified
Applications
E IF IHC WB
Crossreactivity
Hu
Accession #
NM_000157, NP_000148
Shipping Temp
Blue Ice
Storage Temp
-20°C

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 encoding the same protein.

Applications
Suitable for use in Immunofluorescence, ELISA, Western Blot and Immunohistochemistry. Other applications not tested.
Recommended Dilutions
Immunofluorescence: 10ug/ml Immunohistochemistry (FFPE): 3ug/ml Optimal dilutions to be determined by the researcher.
Amino Acid Sequence
SYFSEEGIGYNIIRVPMASCDFSIRTYTYADTPDDFQLHNFSLPEEDTKLKIPLIHRALQLAQRPVSLLASPWTSPTWLKTNGAVNGKGS
Storage and Stability
May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Immunogen
Partial recombinant protein corresponding to aa146-235 from human GBA with GST tag. MW of the GST tag alone is 26kD.
Form
Supplied as a liquid in PBS, pH 7.4.
Purity
Purified by Protein A affinity chromatography.
Specificity
Recognizes human GBA.
References
1. Characterization of the ERAD process of the L444P mutant glucocerebrosidase variant. Bendikov-Bar I, Ron I, Filocamo M, Horowitz M.Blood Cells Mol Dis. 2010 Nov 22. [Epub ahead of print] 2. Characterization of Gaucher disease bone marrow mesenchymal stromal cells reveals an altered inflammatory secretome. Campeau PM, Rafei M, Boivin MN, Sun Y, Grabowski GA, Galipeau J.Blood. 2009 Oct 8;114(15):3181-90. Epub 2009 Jul 8.
USBio References
No references available
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