LYAG/GAA Antibody

Images

 
Western Blot: LYAG/GAA Antibody [H00002548-D01P] - Analysis of GAA expression in mouse intestine.
Western Blot: LYAG/GAA Antibody [H00002548-D01P] - Analysis of GAA expression in transfected 293T cell line by GAA polyclonal antibody.Lane 1: GAA transfected lysate(105.30 KDa).Lane 2: Non-transfected lysate.

Product Details

Summary
Reactivity Hu, MuSpecies Glossary
Applications WB
Clonality
Polyclonal
Host
Rabbit
Conjugate
Unconjugated

Order Details

LYAG/GAA Antibody Summary

Immunogen
GAA (P10253, 1 a.a. - 952 a.a.) full-length human protein. MGVRHPPCSHRLLAVCALVSLATAALLGHILLHDFLLVPRELSGSSPVLEETHPAHQQGASRPGPRDAQAHPGRPRAVPTQCDVPPNSRFDCAPDKAITQEQCEARGCCYIPAKQGLQGAQMGQPWCFFPPSYPSYKLENLSSSEMGYTATLTRTTPTFFPKDILTLRLDVMMETENRLHFTIKDPANRRYEVPLETPRVHSRAPSPLYSVEFSEEPFGVIVHRQLDGRVLLNTTVAPLFFADQFLQLSTSLPSQYITGLAEHLSPLMLSTSWTRITLWNRDLAPTPGANLYGSHPFYLALEDGGSAHGVFLLNSNAMDVVLQPSPALSWRSTGGILDVYIFLGPEPKSVVQQYLDVVGYPFMPPYWGLGFHLCRWGYSSTAITRQVVENMTRAHFPLDVQWNDLDYMDSRRDFTFNKDGFRDFPAMVQELHQGGRRYMMIVDPAISSSGPAGSYRPYDEGLRRGVFITNETGQPLIGKVWPGSTAFPDFTNPTALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIRGSEDGCPNNELENPPYVPGVVGGTLQAATICASSHQFLSTHYNLHNLYGLTEAIASHRALVKARGTRPFVISRSTFAGHGRYAGHWTGDVWSSWEQLASSVPEILQFNLLGVPLVGADVCGFLGNTSEELCVRWTQLGAFYPFMRNHNSLLSLPQEPYSFSEPAQQAMRKALTLRYALLPHLYTLFHQAHVAGETVARPLFLEFPKDSSTWTVDHQLLWGEALLITPVLQAGKAEVTGYFPLGTWYDLQTVPIEALGSLPPPPAAPREPAIHSEGQWVTLPAPLDTINVHLRAGYIIPLQGPGLTTTESRQQPMALAVALTKGGEARGELFWDDGESLEVLERGAYTQVIFLARNNTIVNELVRVTSEGAGLQLQKVTVLGVATAPQQVLSNGVPVSNFTYSPDTKVLDICVSLLMGEQFLVSWC
Specificity
GAA - glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II),
Isotype
IgG
Clonality
Polyclonal
Host
Rabbit
Gene
GAA
Purity
Protein A purified
Innovator's Reward
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Applications/Dilutions

Dilutions
  • Western Blot

Packaging, Storage & Formulations

Storage
Aliquot and store at -20C or -80C. Avoid freeze-thaw cycles.
Buffer
PBS (pH 7.4)
Preservative
No Preservative
Purity
Protein A purified

Notes

Quality control test: Antibody reactive against mammalian transfected lysate.

This product is produced by and distributed for Abnova, a company based in Taiwan.

Alternate Names for LYAG/GAA Antibody

  • Acid alpha-Glucosidase
  • Acid Maltase
  • Aglucosidase alfa
  • EC 3.2.1.20
  • GAA
  • glucosidase, alpha; acid
  • LYAG
  • Lysosomal alphaGlucosidase
  • Lysosomal alpha-Glucosidase

Background

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]

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

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Video Protocols

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Secondary Antibodies

 

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Bioinformatics

Gene Symbol GAA
Entrez
Uniprot