GAA Antibody / Glucosidase alpha acid
| Code | Size | Price |
|---|
| NSJ-R32608-100ug | 100 ug | £535.00 |
Quantity:
Prices exclude any Taxes / VAT
Overview
Host Type: Rabbit
Antibody Isotype: Rabbit IgG
Antibody Clonality: Polyclonal
Regulatory Status: RUO
Target Species:
- Human
- Rat
Applications:
- Immunohistochemistry- Paraffin Embedded (IHC-P)
- Western Blot (WB)
Storage:
After reconstitution, the GAA antibody can be stored for up to one month at 40. For long-term, aliquot and store at -200. Avoid repeated freezing and thawing.
Documents
Further Information
Application Details :
Western blot: 0.5-1ug/ml,Immunohistochemistry (FFPE): 1-2ug/ml
Application Note:
Optimal dilution of the GAA antibody should be determined by the researcher.
Buffer:
Lyophilized from 1X PBS with 2.5% BSA and 0.025% sodium azide
Description:
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.
Format :
Antigen affinity purified
Formulation :
0.5mg/ml if reconstituted with 0.2ml sterile DI water
Immunogen:
Amino acids 494-527 (TALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIR-human) were used as the immunogen for the GAA antibody.
Limitation:
This GAA antibody is available for research use only.
Localization:
Cytoplasmic, membranous
Purity:
Antigen affinity
Species Reactivity :
Human
Uniprot #:
P10253