SAB5702125

Anti-GAA Antibody, clone 5L2L10, Rabbit Monoclonal

• Synonym(s): GAA, LYAG, glucosidase alpha, acid
• UNSPSC Code: 12352203
• NACRES: NA.46

Properties

• biological source: rabbit
• Quality Level: 100
• material: colorless
• clone: 5L2L10, monoclonal
• form: liquid
• species reactivity: rat, rat, human
• concentration: 0.8 mg/mL
• technique(s): western blot: 1:500 - 1:2000
• color: colorless
• isotype: IgG
• immunogen sequence: QEQCEARGCCYIPAKQGLQGAQMGQPWCFFPPSYPSYKLENLSSSEMGYTATLTRTTPTFFPKDILTLRLDVMMETENRLHFTIKDPANRRYEVPLETPHV
• UniProt accession no.: P10253
• shipped in: wet ice
• storage temp.: −20°C
• target post-translational modification: unmodified
• Gene Information: human ... GAA(2548)

Description

General description

This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. 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. Alternative splicing results in multiple transcript variants.

Immunogen

A synthetic peptide corresponding to a sequence within amino acids 100-200 of human GAA (P10253).

PBS with 0.02% sodium azide,0.05% BSA,50% glycerol,pH7.3.

Application

WB

Safety Information

• Storage Class Code: 10 - Combustible liquids
• WGK: WGK 1