Glucosidase, Beta, Acid (GBA) antibody

Antigen: Glucosidase, Beta, Acid (GBA)

Synonyms: GCB, GBA1, GLUC, GC, GCase, betaGC, MGC188451, BETA GLUCOSIDASE 25, BETA-GLUCOSIDASE, BGLU25, T12J13.8, T12J13_8

Clonality: Monoclonal (2E2)

Host: Mouse

Reactivity: Human

Conjugate: Un-conjugated

Application: ELISA, Immunofluorescence (IF), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Western Blotting (WB)

Certificates: ISO 9001:2008

Catalog no.: ABIN394481

Additional Information

Alternative name: GBA

Gene ID: 2629

UniProt: NP_000148

Immunogen: GBA (NP_000148, 146 a.a. ~ 236 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 K

Isotype: IgG2a kappa

Clone: 2E2

Description: Other names: GBA1, GCB, GLUC glucosidase, beta, acid (includes glucosylceramidase)

Characteristics: Purified Mouse Monoclonal Antibody (Mab)

Specificity: GBA (NP_000148, 146 a.a. ~ 236 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.

Molecular Weight: 59716 DA

Comments:

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.

Application Details

Application Notes: ELISA ~~ 1ug/ml~3ng/ml Western blot ~~ 1:500~1000 Immunohistochemistry ~~ 1.5ug/ml Immunofluorescence

Concentration: 0.5 mg/ml

Purification: Purified

Buffer: Clear, colorless solution in phosphate buffered saline, pH 7.2 .

Storage: Maintain refrigerated at 2-8 deg C for up to 6 months. For long term storage store at -20 deg C in small aliquots to prevent freeze-thaw cycles

Research Area: Cell Structure, Metabolism

Restrictions: For Research Use only

Images

Immunofluorescence of monoclonal antibody to GBA (AT2167a) on HeLa cell . [antibody concentration 10 ug/ml] Western Blot analysis of GBA expression in transfected 293T cell line by GBA monoclonal antibody (M01), clone 2E2. Lane 1: GBA transfected lysate(60 KDa). Lane 2: Non-transfected lysate.

anti-Glucosidase, Beta, Acid (GBA) antibody (Image 2)

Publications

Product: Velayati, Yu, Sidransky: "The role of glucocerebrosidase mutations in Parkinson disease and Lewy body disorders." in: Current neurology and neuroscience reports, Vol. 10, Issue 3, pp. 190-8, 2010 (PubMed).

Hu, Xi, Guo et al.: "Association of the glucocerebrosidase N370S allele with Parkinson's disease in two separate Chinese Han populations of mainland China." in: European journal of neurology : the official journal of the European Federation of Neurological Societies, Vol. 17, Issue 12, pp. 1476-8, 2010 (PubMed).

Bailey, Xie, Do et al.: "Variation at the NFATC2 locus increases the risk of thiazolidinedione-induced edema in the Diabetes REduction Assessment with ramipril and rosiglitazone Medication (DREAM) study." in: Diabetes Care, Vol. 33, Issue 10, pp. 2250-3, 2010 (PubMed).

Jeong, Park, Kim: "Clinical and genetic characteristics of Korean patients with Gaucher disease." in: Blood cells, molecules & diseases, Vol. 46, Issue 1, pp. 11-4, 2011 (PubMed).

Dos Santos, Pestana, Diniz et al.: "Mutational analysis of GIGYF2, ATP13A2 and GBA genes in Brazilian patients with early-onset Parkinson's disease." in: Neuroscience letters, Vol. 485, Issue 2, pp. 121-4, 2010 (PubMed).