Glucosylceramidase/GBA Antibody (812201) [Alexa Fluor® 350]

• Reactivity: Hu
• Applications: WB, ICC/IF, KO
• Clone: 812201
• Clonality: Monoclonal
• Host: Mouse
• Conjugate: Alexa Fluor 350

Glucosylceramidase/GBA Antibody (812201) [Alexa Fluor® 350] Summary

• 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.
• Isotype: IgG1
• Clonality: Monoclonal
• Host: Mouse
• Gene: GBA1
• Purity Statement: Protein A or G purified

Applications/Dilutions

• Dilutions:
  • Immunocytochemistry
  • Knockout Validated
  • Western Blot

Packaging, Storage & Formulations

• Storage: Protect from light. Do not freeze. 12 months from date of receipt, 2 to 8 °C as supplied
• Buffer: Supplied 0.2mg/ml in 1X PBS with RDF1 and 0.09% Sodium Azide

Notes

This product is produced by and ships from R&D Systems, Inc., a Bio-Techne brand.

Alternate Names for Glucosylceramidase/GBA Antibody (812201) [Alexa Fluor® 350]

• 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

Background

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).

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

Bioinformatics

• Gene Symbol: GBA1