Applications: | IHC-P-Leica, WB, IHC |
Species Reactivity: | Human |
Isotype: | Rabbit IgG |
Purification: | Affinity Purified Rabbit Polyclonal Antibody |
Description: | This gene encodes acid alpha-glucosidase, which isessential for the degradation of glycogen to glucose in lysosomes.Different forms of acid alpha-glucosidase are obtained byproteolytic processing. Defects in this gene are the cause ofglycogen storage disease II, also known as Pompe's disease, whichis an autosomal recessive disorder with a broad clinical spectrum.Three transcript variants encoding the same protein have been foundfor this gene. |
Further Information: | This GAA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 174-203 amino acids from the N-terminal region of human GAA. |
Synonym: | Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76 kDa lysosomal alpha-glucosidase, 70 kDa lysosomal alpha-glucosidase, GAA |
Ascension Number: | P10253 |
NCBI EntrezGene: | 2548 |
Shipping Conditions: | Ship on cold packs |
Storage: | Maintain refrigerated at 2-8 C for up to 2 weeks. For long term storage store at -20 C in small aliquots to prevent freeze-thaw cycles. |
Usage: | Research use only |