GAA DNAxPab
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More Files
- More Functions
Western Blot (Transfected lysate)
Western Blot analysis of GAA expression in transfected 293T cell line by GAA DNAxPab polyclonal antibody.
Lane 1: GAA transfected lysate(118.25 KDa).
Lane 2: Non-transfected lysate.
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Specification
Product Description
Rabbit polyclonal antibody raised against a full-length human GAA DNA using DNAx™ Immune technology.
Technology
Immunogen
GAA (NP_000143.1, 70 a.a. ~ 952 a.a) full-length human DNA
Sequence
AHPGRPRAVPTQCDVPPNSRFDCAPDKAITQEQCEARGCCYIPAKQGLQGAQMGQPWCFFPPSYPSYKLENLSSSEMGYTATLTRTTPTFFPKDILTLRLDVMMETENRLHFTIKDPANRRYEVPLETPRVHSRAPSPLYSVEFSEEPFGVIVHRQLDGRVLLNTTVAPLFFADQFLQLSTSLPSQYITGLAEHLSPLMLSTSWTRITLWNRDLAPTPGANLYGSHPFYLALEDGGSAHGVFLLNSNAMDVVLQPSPALSWRSTGGILDVYIFLGPEPKSVVQQYLDVVGYPFMPPYWGLGFHLCRWGYSSTAITRQVVENMTRAHFPLDVQWNDLDYMDSRRDFTFNKDGFRDFPAMVQELHQGGRRYMMIVDPAISSSGPAGSYRPYDEGLRRGVFITNETGQPLIGKVWPGSTAFPDFTNPTALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIRGSEDGCPNNELENPPYVPGVVGGTLQAATICASSHQFLSTHYNLHNLYGLTEAIASHRALVKARGTRPFVISRSTFAGHGRYAGHWTGDVWSSWEQLASSVPEILQFNLLGVPLVGADVCGFLGNTSEELCVRWTQLGAFYPFMRNHNSLLSLPQEPYSFSEPAQQAMRKALTLRYALLPHLYTLFHQAHVAGETVARPLFLEFPKDSSTWTVDHQLLWGEALLITPVLQAGKAEVTGYFPLGTWYDLQTVPIEALGSLPPPPAAPREPAIHSEGQWVTLPAPLDTINVHLRAGYIIPLQGPGLTTTESRQQPMALAVALTKGGEARGELFWDDGESLEVLERGAYTQVIFLARNNTIVNELVRVTSEGAGLQLQKVTVLGVATAPQQVLSNGVPVSNFTYSPDTKVLDICVSLLMGEQFLVSWC
Host
Rabbit
Reactivity
Human
Purification
Protein A
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 by GAA DNAxPab polyclonal antibody.
Lane 1: GAA transfected lysate(118.25 KDa).
Lane 2: Non-transfected lysate.
Immunofluorescence (Transfected cell)
Flow Cytometry (Transfected cell)
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Gene Info — GAA
Entrez GeneID
2548GeneBank Accession#
NM_000152.2Protein Accession#
NP_000143.1Gene 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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