SAB1405836

Anti-GAA antibody produced in mouse

purified immunoglobulin, buffered aqueous solution

• Sinônimo(s): LYAG
• Código UNSPSC: 12352203
• NACRES: NA.41

Propriedades

• fonte biológica: mouse
• conjugado: unconjugated
• forma do anticorpo: purified immunoglobulin
• tipo de produto de anticorpo: primary antibodies
• clone: polyclonal
• forma: buffered aqueous solution
• peso molecular: antigen ~105.3 kDa
• reatividade de espécies: human
• técnica(s): western blot: 1 μg/mL
• nº de adesão NCBI: NM_000152.2
• nº de adesão UniProt: P10253
• Condições de expedição: dry ice
• temperatura de armazenamento: −20°C
• modificação pós-traducional do alvo: unmodified
• Informações sobre genes: human ... GAA(2548)

Descrição

Descrição geral

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)

Imunogênio

GAA (P10253, 1 a.a. ~ 952 a.a) full-length human protein.

Sequence
MGVRHPPCSHRLLAVCALVSLATAALLGHILLHDFLLVPRELSGSSPVLEETHPAHQQGASRPGPRDAQAHPGRPRAVPTQCDVPPNSRFDCAPDKAITQEQCEARGCCYIPAKQGLQGAQMGQPWCFFPPSYPSYKLENLSSSEMGYTATLTRTTPTFFPKDILTLRLDVMMETENRLHFTIKDPANRRYEVPLETPRVHSRAPSPLYSVEFSEEPFGVIVHRQLDGRVLLNTTVAPLFFADQFLQLSTSLPSQYITGLAEHLSPLMLSTSWTRITLWNRDLAPTPGANLYGSHPFYLALEDGGSAHGVFLLNSNAMDVVLQPSPALSWRSTGGILDVYIFLGPEPKSVVQQYLDVVGYPFMPPYWGLGFHLCRWGYSSTAITRQVVENMTRAHFPLDVQWNDLDYMDSRRDFTFNKDGFRDFPAMVQELHQGGRRYMMIVDPAISSSGPAGSYRPYDEGLRRGVFITNETGQPLIGKVWPGSTAFPDFTNPTALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIRGSEDGCPNNELENPPYVPGVVGGTLQAATICASSHQFLSTHYNLHNLYGLTEAIASHRALVKARGTRPFVISRSTFAGHGRYAGHWTGDVWSSWEQLASSVPEILQFNLLGVPLVGADVCGFLGNTSEELCVRWTQLGAFYPFMRNHNSLLSLPQEPYSFSEPAQQAMRKALTLRYALLPHLYTLFHQAHVAGETVARPLFLEFPKDSSTWTVDHQLLWGEALLITPVLQAGKAEVTGYFPLGTWYDLQTVPIEALGSLPPPPAAPREPAIHSEGQWVTLPAPLDTINVHLRAGYIIPLQGPGLTTTESRQQPMALAVALTKGGEARGELFWDDGESLEVLERGAYTQVIFLARNNTIVNELVRVTSEGAGLQLQKVTVLGVATAPQQVLSNGVPVSNFTYSPDTKVLDICVSLLMGEQFLVSWC

forma física

Solution in phosphate buffered saline, pH 7.4

Exoneração de responsabilidade

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Informações de segurança

• Código de classe de armazenamento: 10 - Combustible liquids
• Classe de risco de água (WGK): WGK 1
• Ponto de fulgor (°F): Not applicable
• Ponto de fulgor (°C): Not applicable