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Key Product Details

Species Reactivity

Human

Applications

Immunocytochemistry, Knockout Validated, Western Blot

Label

Alexa Fluor 750 (Excitation = 749 nm, Emission = 775 nm)

Antibody Source

Monoclonal Mouse IgG1 Clone # 812201

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

Applications

Application
Recommended Usage

Immunocytochemistry

Optimal dilution of this antibody should be experimentally determined.

Knockout Validated

Optimal dilution of this antibody should be experimentally determined.

Western Blot

Optimal dilution of this antibody should be experimentally determined.

Formulation, Preparation, and Storage

Purification

Protein A or G purified

Formulation

Supplied 0.2mg/ml in 1X PBS with RDF1 and 0.09% Sodium Azide

Shipping

The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below.

Stability & Storage

Protect from light. Do not freeze. 12 months from date of receipt, 2 to 8 °C as supplied

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).

Alternate Names

Alglucerase, GBA, GBA1, Imiglucerase

Entrez Gene IDs

2629 (Human); 14466 (Mouse)

Gene Symbol

GBA

UniProt

Additional Glucosylceramidase/GBA Products

Product Documents

Certificate of Analysis

To download a Certificate of Analysis, please enter a lot number in the search box below.

Note: Certificate of Analysis not available for kit components.

Product Specific Notices


This product is provided under an agreement between Life Technologies Corporation and R&D Systems, Inc, and the manufacture, use, sale or import of this product is subject to one or more US patents and corresponding non-US equivalents, owned by Life Technologies Corporation and its affiliates. The purchase of this product conveys to the buyer the non-transferable right to use the purchased amount of the product and components of the product only in research conducted by the buyer (whether the buyer is an academic or for-profit entity). The sale of this product is expressly conditioned on the buyer not using the product or its components (1) in manufacturing; (2) to provide a service, information, or data to an unaffiliated third party for payment; (3) for therapeutic, diagnostic or prophylactic purposes; (4) to resell, sell, or otherwise transfer this product or its components to any third party, or for any other commercial purpose. Life Technologies Corporation will not assert a claim against the buyer of the infringement of the above patents based on the manufacture, use or sale of a commercial product developed in research by the buyer in which this product or its components was employed, provided that neither this product nor any of its components was used in the manufacture of such product. For information on purchasing a license to this product for purposes other than research, contact Life Technologies Corporation, Cell Analysis Business Unit, Business Development, 29851 Willow Creek Road, Eugene, OR 97402, Tel: (541) 465-8300. Fax: (541) 335-0354.

For research use only

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