Invitrogen™ GAA Polyclonal Antibody

Rabbit Polyclonal Antibody

Supplier:  Invitrogen™ PA5117464

Catalog No. PIPA5117464

Description

This antibody has been tested in direct-ELISA.

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 G
• Regulatory Status: RUO
• Gene ID (Entrez): 14387, 2548
• Content And Storage: -20°C or -80°C if preferred
• Form: Liquid

• Applications: Immunohistochemistry (Paraffin), Western Blot, Immunocytochemistry
• Concentration: 1 mg/mL
• Formulation: PBS with 50% glycerol and 0.03% ProClin 300; pH 7.4
• Gene Accession No.: P10253, P70699
• Gene Symbols: Gaa
• Immunogen: Recombinant Human Lysosomal alpha-glucosidase protein (601-952aa).
• Quantity: 100 μg
• Primary or Secondary: Primary
• Target Species: Human, Mouse
• Product Type: Antibody
• Isotype: IgG

Safety and Handling

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