Full Product Name
Anti-GAA Antibody
Product Gene Name
anti-GAA antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Purity/Purification
Affinity Purification
Concentration
1mg/ml (lot specific)
Immunogen
Recombinant protein of human GAA
Storage Buffer
PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Preparation and Storage
Store at -20 degree C. Avoid freeze/thaw cycles.
Other Notes
Small volumes of anti-GAA antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
anti-GAA antibody
This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing.
Applications Tested/Suitable for anti-GAA antibody
Western Blot (WB)
Application Notes for anti-GAA antibody
WB: 1:500-1:2000
NCBI/Uniprot data below describe general gene information for GAA. It may not necessarily be applicable to this product.
NCBI Accession #
AKI71404.1
[Other Products]
UniProt Secondary Accession #
Q09GN4; Q14351; Q16302; Q8IWE7[Other Products]
UniProt Related Accession #
P10253[Other Products]
Molecular Weight
105,324 Da
NCBI Official Full Name
GAA, partial
NCBI Official Synonym Full Names
glucosidase alpha, acid
NCBI Official Symbol
GAA??[Similar Products]
NCBI Official Synonym Symbols
LYAG
??[Similar Products]
NCBI Protein Information
lysosomal alpha-glucosidase
UniProt Protein Name
Lysosomal alpha-glucosidase
UniProt Synonym Protein Names
Acid maltase; Aglucosidase alfa
Protein Family
Lysosomal alpha-glucosidase
UniProt Gene Name
GAA??[Similar Products]
NCBI Summary for GAA
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. [provided by RefSeq, Jan 2016]
UniProt Comments for GAA
Essential for the degradation of glycogen in lysosomes (PubMed:1856189, PubMed:7717400, PubMed:14695532, PubMed:18429042). Has highest activity on alpha-1,4-linked glycosidic linkages, but can also hydrolyze alpha-1,6-linked glucans (PubMed:29061980).
Research Articles on GAA
1. The narrow substrate-binding pocket of rhGAA is located near the C-terminal ends of beta-strands of the catalytic (beta/alpha)8 domain and shaped by a loop from the N-terminal beta-sheet domain and both inserts I and II.
Precautions
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Disclaimer
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