GAA purified MaxPab mouse polyclonal antibody (B01P)
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More Files
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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(104.72 KDa).
Lane 2: Non-transfected lysate.
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Specification
Product Description
Mouse 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
Mouse
Reactivity
Human
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 (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(104.72 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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Uptake of moss-derived human recombinant GAA in Gaa -/- mice.
Stefan Hintze, Paulina Dabrowska-Schlepp, Birgit Berg, Alexandra Graupner, Andreas Busch, Andreas Schaaf, Benedikt Schoser, Peter Meinke.
JIMD Reports 2021 Feb; 59(1):81.
Application：WB-Ti, Mouse, Mouse quadriceps muscle.
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Uptake of moss-derived human recombinant GAA in Gaa -/- mice.
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