Caspase-6 Activity in a BACHD Mouse Modulates Steady-State Levels of Mutant Huntingtin Protein But Is Not Necessary for Production of a 586 Amino Acid Proteolytic Fragment

Huntington's disease (HD) is caused by a mutation in the huntingtin (htt) gene encoding an expansion of glutamine repeats at the N terminus of the Htt protein. Proteolysis of Htt has been identified as a critical pathological event in HD models. In particular, it has been postulated that proteo...

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Main Authors: Gafni, Juliette, Papanikolaou, Theodora, DeGiacomo, Francesco, Holcomb, Jennifer, Chen, Sylvia, Menalled, Liliana, Kudwa, Andrea, Fitzpatrick, Jon, Miller, Sam, Ramboz, Sylvie, Tuunanen, Pasi I., Lehtimäki, Kimmo K., Yang, X. William, Park, Larry, Kwak, Seung, Howland, David, Park, Hyunsun, Ellerby, Lisa M.
Format: Artigo
Sprog:Inglês
Udgivet: Society for Neuroscience 2012
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Articles
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC3454486/
https://ncbi.nlm.nih.gov/pubmed/22649225
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1523/JNEUROSCI.6379-11.2012
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