GAA, also known as LYAG (Lysosomal alpha-glucosidase), is a crucial enzyme that plays a significant role in lysosomes. It is involved in the breakdown of glycogen, ensuring that it is properly degraded into glucose molecules to maintain normal cellular energy metabolism and other biological processes. However, defects or dysfunction in lysosomal alpha-glucosidase can trigger a series of genetic diseases, the most representative of which is Pompe disease. Pompe disease is an autosomal recessive genetic disease caused by a lack or insufficient function of this enzyme, manifesting as glycogen accumulation in lysosomes, leading to clinical symptoms such as muscle weakness and cardiac hypertrophy.
The following GAA reagents supplied by CUSABIO are manufactured under a strict quality control system. Multiple applications have been validated and solid technical support is offered.
GAA Antibodies for Homo sapiens (Human)
Code | Product Name | Species Reactivity | Application |
---|---|---|---|
CSB-PA009125GA01HU | GAA Antibody |
Human,Mouse,Rat | ELISA,WB,IHC |
CSB-PA009125LA01HU | GAA Antibody |
Human, Mouse | ELISA, WB, IHC, IF |
CSB-PA009125LB01HU | GAA Antibody, HRP conjugated |
Human | ELISA |
CSB-PA009125LC01HU | GAA Antibody, FITC conjugated |
Human | |
CSB-PA009125LD01HU | GAA Antibody, Biotin conjugated |
Human | ELISA |
CSB-RA566370A0HU | GAA Recombinant Monoclonal Antibody |
Human | ELISA, IHC |
GAA Proteins for Homo sapiens (Human)
Code | Product Name | Source |
---|---|---|
CSB-YP009125HU CSB-EP009125HU CSB-BP009125HU CSB-MP009125HU CSB-EP009125HU-B |
Recombinant Human Lysosomal alpha-glucosidase (GAA), partial |
Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
GAA ELISA Kit for Homo sapiens (Human)
Code | Product Name | Sample Types | Sensitivity |
---|---|---|---|
CSB-EL009125HU | Human Lysosomal alpha-glucosidase(GAA) ELISA kit |
serum, plasma, tissue homogenates, cell lysates | 6.25 pg/mL |