Glucosylceramidase/GBA Antibody (812201) [Janelia Fluor® 646]

• Reactivity: Hu
• Applications: WB, ICC/IF, KO
• Clone: 812201
• Clonality: Monoclonal
• Host: Mouse
• Conjugate: Janelia Fluor 646

Glucosylceramidase/GBA Antibody (812201) [Janelia Fluor® 646] 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: GBA
• Purity: Protein A or G purified from hybridoma culture supernatant

Applications/Dilutions

• Dilutions:
  • Immunocytochemistry
  • Knockout Validated
  • Western Blot
• Application Notes: Optimal dilution of this antibody should be experimentally determined.

Packaging, Storage & Formulations

• Storage: Store at 4C in the dark.
• Buffer: 50mM Sodium Borate
• Preservative: 0.05% Sodium Azide
• Purity: Protein A or G purified from hybridoma culture supernatant

Notes

Sold under license from the Howard Hughes Medical Institute, Janelia Research Campus.

Alternate Names for Glucosylceramidase/GBA Antibody (812201) [Janelia Fluor® 646]

• 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

GBA 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 encoding the same protein.

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: GBA