GAA

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.

Popular Product:

GAA Recombinant Monoclonal Antibody

Validated Data

IHC

IHC image of CSB-RA566370A0HU diluted at 1:50 and staining in paraffin-embedded human breast cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.15% DAB.

IHC

IHC image of CSB-RA566370A0HU diluted at 1:50 and staining in paraffin-embedded human placenta tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.15% DAB.

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

GAA Proteins

GAA ELISA Kits

GAA Antibodies

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

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

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

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Popular Pathways

Apoptosis

Breast Cancer

Autophagy

Hippo Signaling Pathway

Wnt Signaling Pathway

Hippo signaling pathway

TGF-beta signaling pathway

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