Recombinant Human GAA Protein, Myc/DDK-tagged, C13 and N15-labeled
Cat.No. : | GAA-4807H |
Product Overview : | GAA MS Standard C13 and N15-labeled recombinant protein (NP_000143) with a C-terminal MYC/DDK tag, was expressed in HEK293 cells. |
- Specification
- Gene Information
- Related Products
- Download
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 : | HEK293 |
Species : | Human |
Tag : | Myc/DDK |
Molecular Mass : | 105.32 kDa |
AA Sequence : | MGVRHPPCSHRLLAVCALVSLATAA LLGHILLHDFLLVPRELSGSSPVLE ETHPAHQQGASRPGPRDAQAHPGRP RAVPTQCDVPPNSRFDCAPDKAITQ EQCEARGCCYIPAKQGLQGAQMGQP WCFFPPSYPSYKLENLSSSEMGYTA TLTRTTPTFFPKDILTLRLDVMMET ENRLHFTIKDPANRRYEVPLETPHV HSRAPSPLYSVEFSEEPFGVIVRRQ LDGRVLLNTTVAPLFFADQFLQLST SLPSQYITGLAEHLSPLMLSTSWTR ITLWNRDLAPTPGANLYGSHPFYLA LEDGGSAHGVFLLNSNAMDVVLQPS PALSWRSTGGILDVYIFLGPEPKSV VQQYLDVVGYPFMPPYWGLGFHLCR WGYSSTAITRQVVENMTRAHFPLDV QWNDLDYMDSRRDFTFNKDGFRDFP AMVQELHQGGRRYMMIVDPAISSSG PAGSYRPYDEGLRRGVFITNETGQP LIGKVWPGSTAFPDFTNPTALAWWE DMVAEFHDQVPFDGMWIDMNEPSNF IRGSEDGCPNNELENPPYVPGVVGG TLQAATICASSHQFLSTHYNLHNLY GLTEAIASHRALVKARGTRPFVISR STFAGHGRYAGHWTGDVWSSWEQLA SSVPEILQFNLLGVPLVGADVCGFL GNTSEELCVRWTQLGAFYPFMRNHN SLLSLPQEPYSFSEPAQQAMRKALT LRYALLPHLYTLFHQAHVAGETVAR PLFLEFPKDSSTWTVDHQLLWGEAL LITPVLQAGKAEVTGYFPLGTWYDL QTVPVEALGSLPPPPAAPREPAIHS EGQWVTLPAPLDTINVHLRAGYIIP LQGPGLTTTESRQQPMALAVALTKG GEARGELFWDDGESLEVLERGAYTQ VIFLARNNTIVNELVRVTSEGAGLQ LQKVTVLGVATAPQQVLSNGVPVSN FTYSPDTKVLDICVSLLMGEQFLVS WCTRTRPLEQKLISEEDLAANDILD YKDDDDKV |
Purity : | > 80% as determined by SDS-PAGE and Coomassie blue staining |
Stability : | Stable for 3 months from receipt of products under proper storage and handling conditions. |
Storage : | Store at -80 centigrade. Avoid repeated freeze-thaw cycles. |
Concentration : | 50 μg/mL as determined by BCA |
Storage Buffer : | 100 mM glycine, 25 mM Tris-HCl, pH 7.3. |
Gene Name : | GAA alpha glucosidase [ Homo sapiens (human) ] |
Official Symbol : | GAA |
Synonyms : | GAA; glucosidase, alpha; acid; lysosomal alpha-glucosidase; glycogen storage disease type II; Pompe disease; acid maltase; aglucosidase alfa; LYAG; |
Gene ID : | 2548 |
mRNA Refseq : | NM_000152 |
Protein Refseq : | NP_000143 |
MIM : | 606800 |
UniProt ID : | P10253 |
Products Types
◆ Recombinant Protein | ||
GAA-2094R | Recombinant Rat GAA Protein, His (Fc)-Avi-tagged | +Inquiry |
Gaa-3124M | Recombinant Mouse Gaa Protein, Myc/DDK-tagged | +Inquiry |
Gaa-164R | Recombinant Rat Gaa Protein, His-tagged | +Inquiry |
GAA-177H | Recombinant Human GAA protein, MYC/DDK-tagged | +Inquiry |
GAA-002H | Active Recombinant Human Acid α-glucosidase (GAA) | +Inquiry |
◆ Lysates | ||
GAA-6077HCL | Recombinant Human GAA 293 Cell Lysate | +Inquiry |
◆ Assay kits | ||
Kit-0353 | GAA Activity Colorimetric Assay Kit | +Inquiry |
Related Gene
For Research Use Only. Not intended for any clinical use. No products from Creative BioMart may be resold, modified for resale or used to manufacture commercial products without prior written approval from Creative BioMart.
Inquiry
- Q&As
- Reviews
Q&As (5)
Ask a questionGAA protein is one of several enzymes involved in glycogen metabolism, each playing a specific role in glycogen breakdown.
Enzyme replacement therapy (ERT) and gene therapy are two treatment options available for Pompe disease patients to replace or correct the deficient GAA protein.
GAA protein deficiency is primarily associated with Pompe disease, but there are other glycogen storage diseases related to deficiencies of different enzymes.
ERT involves the infusion of recombinant GAA protein to replace the missing or deficient enzyme in Pompe disease patients.
While GAA protein therapy can improve the symptoms and quality of life for Pompe disease patients, it may not fully reverse the damage caused by the disease.
Customer Reviews (3)
Write a reviewAdditionally, cost-effectiveness, competitive pricing, and bulk purchase options can also be advantageous for researchers working within limited budgets.
The ability to customize the GAA protein variants or modifications offered by the manufacturer is also advantageous, as it allows me to tailor the protein to my specific research objectives.
I appreciate the manufacturer's commitment to maintaining exceptional protein quality standards, which ensures reliable and consistent results in my trials.
Ask a Question for All GAA Products
Required fields are marked with *
My Review for All GAA Products
Required fields are marked with *
Inquiry Basket