Small, Seeding-Competent Huntingtin Fibrils Are Prominent Aggregate Species in Brains of zQ175 Huntington’s Disease Knock-in Mice

The deposition of mutant huntingtin (mHTT) protein aggregates in neurons of patients is a pathological hallmark of Huntington’s disease (HD). Previous investigations in cell-free and cell-based disease models showed mHTT exon-1 (mHTTex1) fragments with pathogenic polyglutamine (polyQ) tracts (>40...

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Gepubliceerd in:Front Neurosci
Hoofdauteurs: Schindler, Franziska, Praedel, Nicole, Neuendorf, Nancy, Kunz, Severine, Schnoegl, Sigrid, Mason, Michael A., Taxy, Bridget A., Bates, Gillian P., Khoshnan, Ali, Priller, Josef, Grimm, Jan, Maier, Marcel, Boeddrich, Annett, Wanker, Erich E.
Formaat: Artigo
Taal:Inglês
Gepubliceerd in: Frontiers Media S.A. 2021
Onderwerpen:
Neuroscience
Online toegang:https://ncbi.nlm.nih.gov/pmc/articles/PMC8257939/
https://ncbi.nlm.nih.gov/pubmed/34239412
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3389/fnins.2021.682172
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