Product name: | LYAG rabbit pAb |
Reactivity: | Human; Mouse;Rat |
Alternative Names: | Lysosomal alpha-glucosidase (EC 3.2.1.20) (Acid maltase) (Aglucosidase alfa) [Cleaved into: 76 kDa lysosomal alpha-glucosidase; 70 kDa lysosomal alpha-glucosidase] |
Source: | Rabbit |
Dilutions: | WB 1:500-2000 |
Immunogen: | Synthesized peptide derived from human LYAG AA range: 432-482 |
Storage: | -20°C/1 year |
Clonality: | Polyclonal |
Isotype: | IgG |
Concentration: | 1 mg/ml |
Observed Band: | 105kD |
GeneID: | 2548 |
Human Swiss-Prot No: | P10253 |
Cellular localization: | Lysosome . Lysosome membrane . |
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. [provided by RefSeq, Jan 2016], |