Description : |
This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. 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. Alternative splicing results in multiple transcript variants. |
Source : |
In Vitro Cell Free System |
Species : |
Human |
Tag : |
GST |
Form : |
50 mM Tris-HCl, 10 mM reduced Glutathione, pH=8.0 in the elution buffer. |
Molecular Mass : |
131.7 kDa |
Protein Length : |
952 amino acids |
AA Sequence : |
MGVRHPPCSHRLLAVCALVSLATAA LLGHILLHDFLLVPRELSGSSPVLE ETHPAHQQGASRPGPRDAQAHPGRP RAVPTQCDVPPNSRFDCAPDKAITQ EQCEARGCCYIPAKQGLQGAQMGQP WCFFPPSYPSYKLENLSSSEMGYTA TLTRTTPTFFPKDILTLRLDVMMET ENRLHFTIKDPANRRYEVPLETPRV HSRAPSPLYSVEFSEEPFGVIVHRQ LDGRVLLNTTVAPLFFADQFLQLST SLPSQYITGLAEHLSPLMLSTSWTR ITLWNRDLAPTPGANLYGSHPFYLA LEDGGSAHGVFLLNSNAMDVVLQPS PALSWRSTGGILDVYIFLGPEPKSV VQQYLDVVGYPFMPPYWGLGFHLCR WGYSSTAITRQVVENMTRAHFPLDV QWNDLDYMDSRRDFTFNKDGFRDFP AMVQELHQGGRRYMMIVDPAISSSG PAGSYRPYDEGLRRGVFITNETGQP LIGKVWPGSTAFPDFTNPTALAWWE DMVAEFHDQVPFDGMWIDMNEPSNF IRGSEDGCPNNELENPPYVPGVVGG TLQAATICASSHQFLSTHYNLHNLY GLTEAIASHRALVKARGTRPFVISR STFAGHGRYAGHWTGDVWSSWEQLA SSVPEILQFNLLGVPLVGADVCGFL GNTSEELCVRWTQLGAFYPFMRNHN SLLSLPQEPYSFSEPAQQAMRKALT LRYALLPHLYTLFHQAHVAGETVAR PLFLEFPKDSSTWTVDHQLLWGEAL LITPVLQAGKAEVTGYFPLGTWYDL QTVPIEALGSLPPPPAAPREPAIHS EGQWVTLPAPLDTINVHLRAGYIIP LQGPGLTTTESRQQPMALAVALTKG GEARGELFWDDGESLEVLERGAYTQ VIFLARNNTIVNELVRVTSEGAGLQ LQKVTVLGVATAPQQVLSNGVPVSN FTYSPDTKVLDICVSLLMGEQFLVS WC |
Applications : |
Enzyme-linked Immunoabsorbent Assay; Western Blot (Recombinant protein); Antibody Production; Protein Array |
Notes : |
Best use within three months from the date of receipt of this protein. |
Storage : |
Store at -80 centigrade. Aliquot to avoid repeated freezing and thawing. |