GAA Rabbit anti-Human, Polyclonal, Bioss

Rabbit Polyclonal Antibody

Supplier:  Bioss BS13254R

Catalog No. 50-198-2743

Description

This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe′s disease, which is an autosomal recessive disorder with a broad clinical spectrum. Three transcript variants encoding the same protein have been found for this gene.

Specifications

• Antigen: GAA
• Classification: Polyclonal
• Conjugate: Unconjugated
• Gene: Gaa
• Gene Alias: 70 kDa lysosomal alpha-glucosidase; 76 kDa lysosomal alpha-glucosidase; acid (Pompe disease, glycogen storage disease type II); acid alpha-glucosidase; Acid maltase; Aglucosidase alfa; E430018M07Rik; Gaa; glucosidase alpha, acid; glucosidase, alpha, acid; glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II); LYAG; Lysosomal alpha-glucosidase
• Host Species: Rabbit
• Purification Method: Protein A
• Regulatory Status: RUO
• Gene ID (Entrez): 2548
• Content And Storage: -20°C
• Form: Liquid

• Applications: Immunofluorescence, Immunohistochemistry (Paraffin), Western Blot
• Concentration: 1 μg/mL
• Formulation: PBS with 1% BSA, 50% glycerol and 0.09% sodium azide; pH
• Gene Accession No.: P10253
• Gene Symbols: Gaa
• Immunogen: KLH conjugated synthetic peptide derived from human GAA/Glucosidase alpha.
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human
• Product Type: Antibody
• Isotype: IgG