Human GAA ELISA Kit (V2LY-0626-LY4843)
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Basic Information
- Pre-coated ELISA plate: 12 wells * 8 detachable strips
- Standard solution: 0.5ml x1
- Standard diluent: 3ml x1
- Streptavidin-HRP: 6ml x1
- Stop solution: 6ml x1
- Substrate solution A: 6ml x1
- Substrate solution B: 6ml x1
- Wash buffer concentrate (25x): 20ml x1
- Biotinylated antibody: 1ml x1
Formulations & Storage [For reference only, actual COA shall prevail!]
Target
Has highest activity on alpha-1,4-linked glycosidic linkages, but can also hydrolyze alpha-1,6-linked glucans (PubMed:29061980).
Diaphragm contraction Source: BHF-UCL
Glucose metabolic process Source: BHF-UCL
Glycogen catabolic process Source: UniProtKB
Heart morphogenesis Source: Ensembl
Locomotory behavior Source: Ensembl
Lysosome organization Source: BHF-UCL
Maltose metabolic process Source: BHF-UCL
Muscle cell cellular homeostasis Source: Ensembl
Neuromuscular process controlling balance Source: Ensembl
Neuromuscular process controlling posture Source: Ensembl
Regulation of the force of heart contraction Source: Ensembl
Sucrose metabolic process Source: BHF-UCL
Tissue development Source: Ensembl
Vacuolar sequestering Source: BHF-UCL
A metabolic disorder with a broad clinical spectrum. The severe infantile form, or Pompe disease, presents at birth with massive accumulation of glycogen in muscle, heart and liver. Cardiomyopathy and muscular hypotonia are the cardinal features of this form whose life expectancy is less than two years. The juvenile and adult forms present as limb-girdle muscular dystrophy beginning in the lower limbs. Final outcome depends on respiratory muscle failure. Patients with the adult form can be free of clinical symptoms for most of their life but finally develop a slowly progressive myopathy.
Phosphorylation of mannose residues ensures efficient transport of the enzyme to the lysosomes via the mannose 6-phosphate receptor.
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Please try the standard protocols which include: protocols, troubleshooting and guide.
Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols
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Custom Antibody Labeling
We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).
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