Human Glucosylceramidase/GBA Antibody Best Seller

R&D Systems, part of Bio-Techne | Catalog # MAB7410

R&D Systems, part of Bio-Techne
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MAB7410
MAB7410-SP
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Key Product Details

Validated by

Knockout/Knockdown

Species Reactivity

Validated:

Human

Cited:

Human

Applications

Validated:

Immunocytochemistry, Knockout Validated, Simple Western, Western Blot

Cited:

Immunohistochemistry, Western Blot

Label

Unconjugated

Antibody Source

Monoclonal Mouse IgG1 Clone # 812201

View all Glucosylceramidase/GBA Antibodies »

Product Specifications

Immunogen

Chinese hamster ovary cell line CHO-derived recombinant human Glucosylceramidase/GBA
Met1-Gln536
Accession # P04062

Specificity

Detects human Glucosylceramidase/GBA in direct ELISAs. In direct ELISAs, no cross-reactivity with recombinant human Cytosolic beta‑Glucosidase/GBA3 is observed.

Clonality

Monoclonal

Host

Mouse

Isotype

IgG1

Scientific Data Images for Human Glucosylceramidase/GBA Antibody

Detection of Human Glucosylceramidase/GBA antibody by Western Blot.

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.
Glucosylceramidase/GBA antibody in HeLa Human Cell Line by Immunocytochemistry (ICC).

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 Northern-Lights™ 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 antibody by Simple WesternTM.

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.
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Applications for Human Glucosylceramidase/GBA Antibody

Application
Recommended Usage

Immunocytochemistry

8-25 µg/mL
Sample: Immersion fixed HeLa human cervical epithelial carcinoma cell line

Knockout Validated

Glucosylceramidase/GBA is specifically detected in HeLa human cervical epithelial carcinoma parental cell line but is not detectable in Glucosylceramidase/GBA knockout HeLa human cervical epithelial carcinoma cell line.

Simple Western

10 µg/mL
Sample: LNCaP human prostate cancer cell line and HEK293T human embryonic kidney cell line

Western Blot

1 µg/mL
Sample: LNCaP human prostate cancer cell line

Formulation, Preparation, and Storage

Purification

Protein A or G purified from hybridoma culture supernatant

Reconstitution

Sterile PBS to a final concentration of 0.5 mg/mL. For liquid material, refer to CoA for concentration.

Reconstitution Buffer Available:
Size / Price
Qty

Formulation

Lyophilized from a 0.2 μm filtered solution in PBS with Trehalose. *Small pack size (SP) is supplied either lyophilized or as a 0.2 µm filtered solution in PBS.

Shipping

Lyophilized product is shipped at ambient temperature. Liquid small pack size (-SP) is shipped with polar packs. Upon receipt, store immediately at the temperature recommended below.

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.

Alternate Names

Alglucerase, GBA, GBA1, Imiglucerase

Entrez Gene IDs

2629 (Human); 14466 (Mouse)

Gene Symbol

GBA

UniProt

P04062

Additional Glucosylceramidase/GBA Products

Product Documents for Human Glucosylceramidase/GBA Antibody

Product Datasheets

Certificate of Analysis

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Product Specific Notices for Human Glucosylceramidase/GBA Antibody

For research use only

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