Human Glucosylceramidase/GBA Antibody MAB7410: R&D Systems

Human Glucosylceramidase/GBA Antibody

  
  • Species Reactivity
    Human
  • Specificity
    Detects human Glucosylceramidase/GBA in direct ELISAs. In direct ELISAs, no cross-reactivity with recombinant human Cytosolic beta‑Glucosidase/GBA3 is observed.
  • Source
    Monoclonal Mouse IgG1 Clone # 812201
  • Purification
    Protein A or G purified from hybridoma culture supernatant
  • Immunogen
    Chinese hamster ovary cell line CHO-derived recombinant human Glucosylceramidase/GBA
    Met1-Gln536
    Accession # P04062
  • Formulation
    Lyophilized from a 0.2 μm filtered solution in PBS with Trehalose. *Small pack size (SP) is supplied as a 0.2 µm filtered solution in PBS.
  • Label
    Unconjugated
Applications
  •  
    Recommended
    Concentration
    Sample
  • Western Blot
    1 µg/mL
    See below
  • Simple Western
    10 µg/mL
    See below
  • Immunocytochemistry
    8-25 µg/mL
    See below
Please Note: Optimal dilutions should be determined by each laboratory for each application. General Protocols are available in the Technical Information section on our website.
Data Examples
Detection of Human Glucosylceramidase/GBA by Western Blot.

Western blot shows lysates of LNCaP human prostate cancer cell line. PVDF membrane was probed with 1 µg/mL of Mouse Anti-Human Glucosylceramidase/GBA Monoclonal Antibody (Catalog # MAB7410) followed by HRP-conjugated Anti-Mouse IgG Secondary Antibody (Catalog # HAF007). A specific band was detected for Glucosylceramidase/GBA at approximately 60 kDa (as indicated). This experiment was conducted under reducing conditions and using Immunoblot Buffer Group 1.

Immunocytochemistry
Glucosylceramidase/GBA in HeLa Human Cell Line.

Glucosylceramidase/GBA was detected in immersion fixed HeLa human cervical epithelial carcinoma cell line using Mouse Anti-Human Glucosylceramidase/GBA Monoclonal Antibody (Catalog # MAB7410) at 15 µg/mL for 3 hours at room temperature. Cells were stained using the Northern­Lights™ 557-conjugated Anti-Mouse IgG Secondary Antibody (red; Catalog # NL007) and counterstained with DAPI (blue). Specific staining was localized to lysosomes. View our protocol for Fluorescent ICC Staining of Cells on Coverslips.

Detection of Human Glucosylceramidase/GBA by Simple WesternTM. Simple Western lane view shows lysates of LNCaP human prostate cancer cell line and HEK293T human embryonic kidney cell line, loaded at 0.2 mg/mL. A specific band was detected for Glucosylceramidase/GBA at approximately 77 kDa (as indicated) using 10 µg/mL of Mouse Anti-Human Glucosylceramidase/GBA Monoclonal Antibody (Catalog # MAB7410). This experiment was conducted under reducing conditions and using the 12-230 kDa separation system.

Preparation and Storage
  • Reconstitution
    Sterile PBS to a final concentration of 0.5 mg/mL.
  • Shipping
    The product is shipped at ambient temperature. Upon receipt, store it immediately at the temperature recommended below. *Small pack size (SP) is shipped with polar packs. Upon receipt, store it immediately at -20 to -70 °C
  • Stability & Storage
    Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
    • 12 months from date of receipt, -20 to -70 °C as supplied.
    • 1 month, 2 to 8 °C under sterile conditions after reconstitution.
    • 6 months, -20 to -70 °C under sterile conditions after reconstitution.
Background: Glucosylceramidase/GBA
Glucosylceramidase is a lysosomal enzyme that cleaves the beta-glucosidic linkage of glucosylceramide (1, 2), an intermediate in glycolipid metabolism. The mature enzyme has 497 amino acids with a molecular weight of 62 kDa (3). Glycosylation occurs at four of five N-glycosylation sites and is essential for the trafficking and activity of the enzyme (4). The enzyme is activated in lysosomes by saposin C, although the mechanism of activation is not well understood (5). Defects in Glucosylceramidase are the cause of Gaucher disease, also known as glucocerebrosidase deficiency (6). Gaucher disease is the most prevalent lysosomal storage disease, characterized by accumulation of glucosylceramide in the reticulo-endothelial system. Symptoms of Gaucher disease may include enlarged spleen and liver, liver malfunction, skeletal disorders and bone lesions, severe neurologic complications, swelling of lymph nodes, anemia, low blood platelets and yellow fatty deposits on the white of the eye (7). Currently, enzyme replacement therapy is used to treat patients with the disease (8, 9).
  • References:
    1. Sorge, J. et al. (1985) Proc. Natl. Acad. Sci. USA 82:7289.
    2. Ginns, E. I. et al. (1984) Biochem. Biophyl. Res. Commun. 123:574.
    3. Horowitz, M. et al. (1989) Genomics 4:87.
    4. Grace, M.E. et al. (1994) J. Biol. Chem. 269:2283.
    5. Bruhn, h. (2005) Biochem. J. 389:249.
    6. Liou, B. et al. (2006) J. Biol. Chem. 281:4242.
    7. Grabowski, G.A. (2008). Lancet 372: 1263–1271.
    8. Zheng, W. et al. (2007) Proc. Natl. Acad. Sci. USA 104:13192.
    9. Beutler, E. and Gelbart, T. (1996) Hum. Mutat. 8:207.
  • Entrez Gene IDs:
    2629 (Human); 14466 (Mouse)
  • Alternate Names:
    Acid beta-glucosidase; Alglucerase; beta-glucocerebrosidase; D-glucosyl-N-acylsphingosine glucohydrolase; EC 3.2.1.45; GBA; GBA1; GC; GCB; GLUC; glucosidase, beta, acid; glucosidase, beta; acid (includes glucosylceramidase); Glucosylceramidase; Imiglucerase; lysosomal glucocerebrosidase
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