GAA purified MaxPab rabbit polyclonal antibody (D01P)
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More Files
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Western Blot (Tissue lysate)
GAA MaxPab rabbit polyclonal antibody. Western Blot analysis of GAA expression in mouse intestine.
Western Blot (Transfected lysate)
Western Blot analysis of GAA expression in transfected 293T cell line (H00002548-T01) by GAA MaxPab polyclonal antibody.
Lane 1: GAA transfected lysate(105.30 KDa).
Lane 2: Non-transfected lysate.
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Specification
Product Description
Rabbit polyclonal antibody raised against a full-length human GAA protein.
Immunogen
GAA (P10253, 1 a.a. ~ 952 a.a) full-length human protein.
Sequence
MGVRHPPCSHRLLAVCALVSLATAALLGHILLHDFLLVPRELSGSSPVLEETHPAHQQGASRPGPRDAQAHPGRPRAVPTQCDVPPNSRFDCAPDKAITQEQCEARGCCYIPAKQGLQGAQMGQPWCFFPPSYPSYKLENLSSSEMGYTATLTRTTPTFFPKDILTLRLDVMMETENRLHFTIKDPANRRYEVPLETPRVHSRAPSPLYSVEFSEEPFGVIVHRQLDGRVLLNTTVAPLFFADQFLQLSTSLPSQYITGLAEHLSPLMLSTSWTRITLWNRDLAPTPGANLYGSHPFYLALEDGGSAHGVFLLNSNAMDVVLQPSPALSWRSTGGILDVYIFLGPEPKSVVQQYLDVVGYPFMPPYWGLGFHLCRWGYSSTAITRQVVENMTRAHFPLDVQWNDLDYMDSRRDFTFNKDGFRDFPAMVQELHQGGRRYMMIVDPAISSSGPAGSYRPYDEGLRRGVFITNETGQPLIGKVWPGSTAFPDFTNPTALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIRGSEDGCPNNELENPPYVPGVVGGTLQAATICASSHQFLSTHYNLHNLYGLTEAIASHRALVKARGTRPFVISRSTFAGHGRYAGHWTGDVWSSWEQLASSVPEILQFNLLGVPLVGADVCGFLGNTSEELCVRWTQLGAFYPFMRNHNSLLSLPQEPYSFSEPAQQAMRKALTLRYALLPHLYTLFHQAHVAGETVARPLFLEFPKDSSTWTVDHQLLWGEALLITPVLQAGKAEVTGYFPLGTWYDLQTVPIEALGSLPPPPAAPREPAIHSEGQWVTLPAPLDTINVHLRAGYIIPLQGPGLTTTESRQQPMALAVALTKGGEARGELFWDDGESLEVLERGAYTQVIFLARNNTIVNELVRVTSEGAGLQLQKVTVLGVATAPQQVLSNGVPVSNFTYSPDTKVLDICVSLLMGEQFLVSWC
Host
Rabbit
Reactivity
Human, Mouse
Quality Control Testing
Antibody reactive against mammalian transfected lysate.
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 (Tissue lysate)
GAA MaxPab rabbit polyclonal antibody. Western Blot analysis of GAA expression in mouse intestine.Western Blot (Transfected lysate)
Western Blot analysis of GAA expression in transfected 293T cell line (H00002548-T01) by GAA MaxPab polyclonal antibody.
Lane 1: GAA transfected lysate(105.30 KDa).
Lane 2: Non-transfected lysate.
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Gene Info — GAA
Entrez GeneID
2548GeneBank Accession#
NM_000152.2Protein Accession#
P10253Gene 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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Moss-Derived Human Recombinant GAA Provides an Optimized Enzyme Uptake in Differentiated Human Muscle Cells of Pompe Disease.
Hintze S, Limmer S, Dabrowska-Schlepp P, Berg B, Krieghoff N, Busch A, Schaaf A, Meinke P, Schoser B.
International Journal of Molecular Sciences 2020 Apr; 21(7):2642.
Application：WB-Re, Recombinant protein.
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Moss-Derived Human Recombinant GAA Provides an Optimized Enzyme Uptake in Differentiated Human Muscle Cells of Pompe Disease.
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