GAA Rabbit mAb

Catalog Number:

77643

Amount:

100μg

Price:

$318

Swiss-Prot No:

P10253

Form of Antibody:

Storage/Stability:

Immunogen:

Recombinant protein of human GAA.

Purification:

Affinity purification

Specificity/Sensitivity:

Applications:

WB

Background:

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.

References:

appl_detail:

WB 1:500 - 1:2000

Datasheet PDF