LYAG/GAA Antibody - Azide and BSA Free

Western Blot: LYAG/GAA Antibody [H00002548-B01P] - Analysis of GAA expression in transfected 293T cell line by GAA polyclonal antibody. Lane 1: GAA transfected lysate(104.72 KDa). Lane 2: Non-transfected lysate.

• Reactivity: Hu
• Applications: WB
• Clonality: Polyclonal
• Host: Mouse
• Conjugate: Unconjugated
• Format: Azide and BSA Free

LYAG/GAA Antibody - Azide and BSA Free Summary

• Description: Quality control test: Antibody reactive against mammalian transfected lysate.
• 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: Mouse
• Gene: GAA
• Purity: Protein A purified

Applications/Dilutions

• Dilutions:
  • Western Blot
• Application Notes: Antibody reactivity against Recombinant Protein with GST tag on ELISA and WB and also on transfected lysate in WB. GST tag alone is used as a negative control.

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

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

Alternate Names for LYAG/GAA Antibody - Azide and BSA Free

• 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.

Publications for LYAG/GAA Antibody (H00002548-B01P)(1)

Publication using H00002548-B01P

• Stefan H, Paulina D, Birgit B et al. Uptake of moss-derived human recombinant GAA in Gaa -/- mice. JIMD Rep. 2021-02-01 [PMID: 33977033]

Bioinformatics

• Gene Symbol: GAA
• Entrez:
  • 2548(Human)
• Uniprot:
  • P10253()