GAA Rabbit mAb (AMR11704N)

Product Specifications

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.

Overview

We constantly strive to ensure we provide our customers with the best antibodies. As a result of this work we offer this antibody in purified format. We are in the process of updating our datasheets. If you have any questions regarding this update, please feel free to contact our technical support team. This product is a high quality GAA Rabbit mAb (APR24466N4) .

Synonyms

GAA; LYAG; glucosidase alpha; acid

Gene ID

2548

UniProt

P10253

Cellular Locus

Lysosome, Lysosome membrane

Dilution

WB 1:500 - 1:2000

Form

Liquid

Buffer

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

Molecular Weight

Calculated MW: 120,100,75kDa Observed MW: 76KDa/105KDa

Storage Conditions

Store at 4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.