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.
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.
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) .
Gene ID URL
https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=2548
Uniprot URL
https://www.uniprot.org/uniprot/P10253
AA Sequence
Email for sequence
Available Sizes
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