Lysosomal alpha-glucosidase is an enzyme that in humans is encoded by the GAA gene. 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.
Formulation
0.5mg/ml if reconstituted with 0.2ml sterile DI water
Host
Mouse
Immunogen Region
Amino acids TALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIR from the human protein were used as the immunogen for the Glucosidase Alpha Acid antibody.
Isotype
IgG2b
Predicted Reactivity
Human
Reactivity
Human
Recombinant
No
Subcellular Location
Cytoplasmic, membranous
Uniprot
P10253
Clone No
2G7
Buffer
Lyophilized from 1X PBS with 2% Trehalose and 0.025% sodium azide
Format
Purified
Purification
Affinity purified
Storage
After reconstitution, the Glucosidase Alpha Acid antibody can be stored for up to one month at 4°C. For long-term, aliquot and store at -20°C. Avoid repeated freezing and thawing.
Applications
WB, IHC-P, IF, FACS
Dilution
Western blot: 0.5-1ug/ml,Immunohistochemistry: 1-2ug/ml,Immunofluorescence: 2-4ug/ml,Flow cytometry: 1-3ug/million cells