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The cytoprotective role of GM1 ganglioside in Huntington disease cells.
| Content Provider | Europe PMC |
|---|---|
| Author | Hart, Hannah S. Valentin, Madeline A. Peters, Stephanie Toering Holler, Susan W. Wang, Hongmin Harmon, Aaron F. Holler, Larry D. |
| Abstract | BackgroundHuntington disease (HD) is a neurodegenerative disease where a genetic mutation leads to excessive polyglutamine (Q) repeats in the huntingtin protein. The polyglutamine repeats create toxic plaques when the protein is cleaved, leading to neuron death. The glycolipid GM1 ganglioside (GM1) has been shown to be neuroprotective in HD models, as it prevents the cleavage of the mutant huntingtin protein by phosphorylation of serine 13 and 16. Previous studies have tested GM1 in both adult-onset and juvenile-onset HD models, but this study set out to investigate whether GM1 mediated cytoprotection is influenced by the length of polyglutamine repeats.Method and resultThis study utilized cell culture to analyze the effect of GM1 on cell viability, directly comparing the response between cells with adult-onset HD and juvenile-onset HD. HEK293 cells expressing either wild-type huntingtin (Htt) (19Q) exon 1, adult-onset HD mutant Htt exon 1 (55Q), or Juvenile HD mutant Htt exon 1 (94Q) were assessed for cell viability using the WST-1 assay. Our results suggested moderate doses of GM1 increased cell viability for all cell lines when compared to untreated cells. When comparing HEK293 55Q and 94Q cells, there was no difference in cell viability within each dose of GM1.ConclusionThese data suggest cellular responses to GM1 are independent of polyglutamine repeats in HD cells and provide insight on GM1’s application as a therapeutic agent for HD and other diseases. |
| Related Links | https://europepmc.org/backend/ptpmcrender.fcgi?accid=PMC9712292&blobtype=pdf |
| ISSN | 03014851 |
| Journal | Molecular Biology Reports [Mol Biol Rep] |
| Volume Number | 49 |
| DOI | 10.1007/s11033-022-07830-2 |
| PubMed Central reference number | PMC9712292 |
| Issue Number | 12 |
| PubMed reference number | 36180805 |
| e-ISSN | 15734978 |
| Language | English |
| Publisher | Springer Netherlands |
| Publisher Date | 2022-09-30 |
| Publisher Place | Dordrecht |
| Access Restriction | Open |
| Rights License | Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. © The Author(s) 2022 |
| Subject Keyword | GM1 ganglioside Huntington’s disease Juvenile Huntington’s disease Mutant huntingtin Polyglutamine repeats Cytoprotection |
| Content Type | Text |
| Resource Type | Article |
| Subject | Genetics Medicine Molecular Biology |
