Glucosidase Alpha Acid Antibody / GAA
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.
Product Specifications
CAS Number
9007-83-4
Specifications
Western blot: 0.5-1 µg/mL, Immunohistochemistry: 1-2 µg/mL, Immunofluorescence: 2-4 µg/mL
UniProt
P10253
Host
Mouse
Reactivity
Human
Immunogen
Amino acids TALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIR from the human protein were used as the immunogen for the Glucosidase Alpha Acid antibody.
Clonality
Monoclonal
Isotype
IgG2b
Clone
2G7
Applications
WB, IHC-P, IF
Purity
Affinity purified
Format
Purified
Buffer
Lyophilized from 1X PBS with 2% Trehalose and 0.025% sodium azide
Reconstitution
Limitations
This Glucosidase Alpha Acid antibody is available for research use only.
Storage Conditions
Formulation
0.5 mg/mL if reconstituted with 0.2ml sterile DI water
Applications Notes
Optimal dilution of the Glucosidase Alpha Acid antibody should be determined by the researcher.
Location
Cytoplasmic, membranous
Image Legend
Frequently Asked Questions
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