GAA, Mouse, Clone: 3C6, Abnova™

Catalog No.	Quantity
89-022-248	100 μg

Catalog No. 89-022-248 Supplier Abnova Corporation Supplier No. H00002548M01

Description

Mouse monoclonal antibody raised against a partial recombinant GAA.

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. [provided by RefSeq]

Sequence: GEARGELFWDDGESLEVLERGAYTQVIFLARNNTIVNELVRVTSEGAGLQLQKVTVLGVATAPQQVLSNGVPVSNFTYSPDTKVLDICVSLLMGEQFLVSWC

Specifications

• Antigen: GAA
• Applications: ELISA, Western Blot
• Classification: Monoclonal
• Clone: 3C6
• Conjugate: Unconjugated
• Description: Mouse monoclonal antibody raised against a partial recombinant GAA.
• Formulation: PBS with no preservative; pH 7.4
• Gene: GAA
• Gene Accession No.: BC040431
• Gene Alias: LYAG
• Gene Symbols: GAA
• Host Species: Mouse
• Immunogen: GAA (AAH40431, 851 a.a. ∼ 952 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
• Purification Method: Affinity chromatography
• Quantity: 100 μg
• Regulatory Status: RUO
• Primary or Secondary: Primary
• Gene ID (Entrez): 2548
• Target Species: Human
• Content And Storage: Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
• Product Type: Antibody
• Form: Liquid
• Isotype: IgG1 κ