MABS1935

Anti-α-galactosidase A Antibody

• Código UNSPSC: 12352203
• NACRES: NA.41

Propriedades

• fonte biológica: mouse
• Nível de qualidade: 200
• conjugado: unconjugated
• forma do anticorpo: purified antibody
• tipo de produto de anticorpo: primary antibodies
• clone: 6G8, monoclonal
• peso molecular: calculated mol wt 48.77 kDa
• reatividade de espécies: human
• embalagem: antibody small pack of 100 μg
• técnica(s): immunocytochemistry: suitable; immunohistochemistry (formalin-fixed, paraffin-embedded sections): suitable; western blot: suitable
• Isotipo: IgG1κ
• nº de adesão UniProt: P06280
• Condições de expedição: dry ice
• temperatura de armazenamento: 2-8°C
• modificação pós-traducional do alvo: unmodified
• Informações sobre genes: human ... GLA(2717)

Descrição

Descrição geral

a-Galactosidase A (UniProt: P06280; EC:3.2.1.22, Alpha-D-galactosidase A, Alpha-D-galactoside galactohydrolase, Galactosylgalactosylglucosylceramidas GLA, Melibiase, Agalsidase) is encoded by the GLA gene (Gene ID: 2717) in human. a-galactosidase A is a homodimeric, lysosomal enzyme that catalyzes the hydrolysis of terminal alpha-galactosyl moieties from glycolipids and glycoproteins and participates to their degradation in the lysosome. It is synthesized with a signal peptide (aa 1-31), which is subsequently cleaved off to generate the mature form that is heavily glycosylated with asparagine-linked high mannose groups. Mutations in GLA gene have been linked to Fabry disease that is characterized by glycolipid accumulation in various tissues and leads to other complication such as renal failure and cardiac and cerebral complications.

Especificidade

Clone 6G8 is a mouse monoclonal antibody that specifically detects human a-Galactosidase A.

Imunogênio

Recombinant full-length human a-Galactosidase A (without the signal peptide).

Aplicação

Quality Control Testing

Evaluated by Immunocytochemistry in Acetone fixed HEK 293 cells..

Immunocytochemistry Analysis: A 1:25 dilution of this antibody detected a-Galactosidase A in acetone fixed HEK 293 cells.

Tested Applications

Immunocytochemistry Analysis: A 1:50 dilution from a representative lot detected a-Galactosidase A in HeLa cells.

Immunohistochemistry Applications: A representative lot detected a-Galactosidase A in Immunohistochemistry applications (Tian, W., et. al. (2019). Nat Commun. 10(1):1785).

Immunocytochemistry Analysis: A representative lot detected a-Galactosidase A in acetone fixed HEK293 cells either HEK293 overexpressing GLA (A), HEK293 wild type (WT; B), or HEK293 cells with KO of GLA (C) (Courtesy of Asst. Prof. Zhang Yang, Ph.D., and Assoc. Prof. Ulla Mandel, Ph.D., Copenhagen Center for Glycomics, University of Copenhagen, Denmark).

Western Blotting Analysis: A representative lot detected a-Galactosidase A in lysates from HEK293 cells overexpressing GLA, HEK293 (WT), but not in HEK293 cells with GLA knockout (Courtesy of Asst. Prof. Zhang Yang, Ph.D., and Assoc. Prof. Ulla Mandel, Ph.D., Copenhagen Center for Glycomics, University of Copenhagen, Denmark).

Note: Actual optimal working dilutions must be determined by end user as specimens, and experimental conditions may vary with the end user

Anti-a-galactosidase A, clone 6G8, Cat. No. MABS1935, is a mouse monoclonal antibody that detects -a-galactosidase A and is tested for use in Immunocytochemistry, Immunohistochemistry, and Western Blotting.

forma física

Purified mouse monoclonal antibody IgG1 in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.

Armazenamento e estabilidade

Stable for 1 year at +2°C to +8°C from date of receipt.

Outras notas

Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.

Exoneração de responsabilidade

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Informações de segurança

• Código de classe de armazenamento: 12 - Non Combustible Liquids
• Classe de risco de água (WGK): WGK 1
• Ponto de fulgor (°F): Not applicable
• Ponto de fulgor (°C): Not applicable