Mouse anti-Human Alpha galactosidase A Monoclonal Antibody | anti-AGAL antibody

Alpha galactosidase A Monoclonal Antibody

Cat. Num. AAA2541810

• Gene Names: GLA; GALA
• Reactivity: Human
• Applications: Western Blot, Immunohistochemistry, Immunofluorescence, ELISA
• Purity: Protein A purification
• Synonyms: Alpha galactosidase A; Monoclonal Antibody; Alpha galactosidase A Monoclonal Antibody; Agalsidase alfa; Alpha D galactosidase A; Alpha D galactoside galactohydrolase 1; Alpha D galactoside galactohydrolase; Alpha gal A; Alpha-D-galactosidase A; Alpha-D-galactoside galactohydrolase; Alpha-galactosidase A; GALA; Galactosidase; alpha; GLA; GLA protein; Melibiase; anti-AGAL antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG2a
• Purity/Purification: Protein A purification
• Form/Format: PBS with 0.02% sodium azide, 50% glycerol, PH7.3
• Sequence Length: 429

• Applicable Applications for anti-AGAL antibody: Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF), ELISA (EIA)
• Application Notes: WB: 1:500-1:5000; IHC: 1:20-1:200; IF: 1:20-1:200
• Immunogen: Fusion protein of Alpha galactosidase A
• Note: Centrifuge before opening to ensure complete recovery of vial contents.
• Preparation and Storage: Store at -20 degree C. Avoid freeze / thaw cycles.

Western Blot (WB)

(Western Blot analysis of HeLa cells using Alpha galactosidase A Monoclonal Antibody at dilution of 1:1000)

Related Product Information for anti-AGAL antibody

GLA, also named as Melibiase, Agalsidase and Alpha-galactosidase A, belongs to the glycosyl hydrolase 27 family. It hydrolyzes terminal, non-reducing alpha-D-galactose residues in alpha-D-galactosides, including galactose oligosaccharides, galactomannans and galactolipids. Fabry disease is an X-linked lysosomal storage disorder resulting from the deficient activity of GLA. Enzyme replacement therapy (ERT) with GLA is currently the most effective therapeutic strategy for patients with Fabry disease, a lysosomal storage disease.

Product Categories/Family for anti-AGAL antibody

Cancer; Cardiovascular

NCBI and Uniprot Product Information

• NCBI GI #: 4504009
• NCBI GeneID: 2717
• NCBI Accession #: NP_000160.1
• NCBI GenBank Nucleotide #: NM_000169.2
• UniProt Accession #: P06280; Q6LER7
• Molecular Weight: Calculated: 49kDa; Observed: 49kDa
• NCBI Official Full Name: alpha-galactosidase A
• NCBI Official Synonym Full Names: galactosidase alpha
• NCBI Official Symbol: GLA
• NCBI Official Synonym Symbols: GALA
• NCBI Protein Information: alpha-galactosidase A
• UniProt Protein Name: Alpha-galactosidase A
• UniProt Gene Name: GLA

NCBI Description

This gene encodes a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. This enzyme predominantly hydrolyzes ceramide trihexoside, and it can catalyze the hydrolysis of melibiose into galactose and glucose. A variety of mutations in this gene affect the synthesis, processing, and stability of this enzyme, which causes Fabry disease, a rare lysosomal storage disorder that results from a failure to catabolize alpha-D-galactosyl glycolipid moieties. [provided by RefSeq, Jul 2008]