Invitrogen™ GAA Polyclonal Antibody

Rabbit Polyclonal Antibody

Brand:  Invitrogen™ PA529051

Product Code. 13403177

Description

Recommended positive controls: 293T, A431, HeLa, HepG2. Store product as a concentrated solution. Centrifuge briefly prior to opening the vial.

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: Antigen affinity chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 2548
• Content And Storage: Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
• Form: Liquid

• Applications: Immunohistochemistry (Paraffin), Western Blot
• Concentration: 0.76 mg/mL
• Formulation: PBS with 20% glycerol and 0.025% ProClin 300; pH 7
• Gene Accession No.: P10253
• Gene Symbols: Gaa
• Immunogen: Recombinant protein encompassing a sequence within the C-terminus region of human LYAG. The exact sequence is proprietary.
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human
• Product Type: Antibody
• Isotype: IgG