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

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 (&g...

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Bibliographic Details
Main Authors: Franziska Schindler, Nicole Praedel, Nancy Neuendorf, Severine Kunz, Sigrid Schnoegl, Michael A. Mason, Bridget A. Taxy, Gillian P. Bates, Ali Khoshnan, Josef Priller, Jan Grimm, Marcel Maier, Annett Boeddrich, Erich E. Wanker
Format: Article
Language:English
Published: Frontiers Media S.A. 2021-06-01
Series:Frontiers in Neuroscience
Subjects:
Huntington’s disease
aggregates
seeding
mHTT
zQ175
brain
Online Access:https://www.frontiersin.org/articles/10.3389/fnins.2021.682172/full

Internet

https://www.frontiersin.org/articles/10.3389/fnins.2021.682172/full

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