GAA monoclonal antibody (M01), clone 3C6
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More Files
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Specification
Product Description
Mouse monoclonal antibody raised against a partial recombinant GAA.
Immunogen
GAA (AAH40431, 851 a.a. ~ 952 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Sequence
GEARGELFWDDGESLEVLERGAYTQVIFLARNNTIVNELVRVTSEGAGLQLQKVTVLGVATAPQQVLSNGVPVSNFTYSPDTKVLDICVSLLMGEQFLVSWC
Host
Mouse
Reactivity
Human
Isotype
IgG1 Kappa
Quality Control Testing
Antibody Reactive Against Recombinant Protein.
Western Blot detection against Immunogen (36.96 KDa) .
Storage Buffer
In 1x PBS, pH 7.4
Storage Instruction
Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
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Applications
Western Blot (Recombinant protein)
Sandwich ELISA (Recombinant protein)
Detection limit for recombinant GST tagged GAA is 0.3 ng/ml as a capture antibody.ELISA
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Gene Info — GAA
Entrez GeneID
2548GeneBank Accession#
BC040431Protein Accession#
AAH40431Gene Name
GAA
Gene Alias
LYAG
Gene Description
glucosidase, alpha; acid
Gene Ontology
HyperlinkGene Summary
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. 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. Three transcript variants encoding the same protein have been found for this gene. [provided by RefSeq
Other Designations
acid alpha-glucosidase|acid maltase|alpha-glucosidase|glycogen storage disease type II|lysosomal alpha-glucosidase
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Interactome
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Pathway
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Disease
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Publication Reference
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Discovery of a novel noniminosugar acid α glucosidase chaperone series.
Xiao J, Westbroek W, Motabar O, Lea WA, Hu X, Velayati A, Zheng W, Southall N, Gustafson AM, Goldin E, Sidransky E, Liu K, Simeonov A, Tamargo RJ, Ribes A, Matalonga L, Ferrer M, Marugan JJ.
Journal of Medicinal Chemistry 2012 Sep; 55(17):7546.
Application:WB, Human, Human fibroblasts, HEK 293.
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Glycosylation-related gene expression is linked to differentiation status in glioblastomas undifferentiated cells.
Cheray M, Petit D, Forestier L, Karayan-Tapon L, Maftah A, Jauberteau MO, Battu S, Gallet FP, Lalloue F.
Cancer Letters 2011 Dec; 312(1):24.
Application:WB-Ce, Human, U87 MG, U251 cells.
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Discovery of a novel noniminosugar acid α glucosidase chaperone series.
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