An Expanded Polyproline Domain Maintains Mutant Huntingtin Soluble in vivo and During Aging

Huntington’s disease is a dominantly inherited neurodegenerative disorder caused by the expansion of a CAG repeat, encoding for the amino acid glutamine (Q), present in the first exon of the protein huntingtin. Over the threshold of Q39 HTT exon 1 (HTTEx1) tends to misfold and aggregate into large i...

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Bibliographic Details
Main Authors: Maria Lucia Pigazzini, Mandy Lawrenz, Anca Margineanu, Gabriele S. Kaminski Schierle, Janine Kirstein
Format: Article
Language:English
Published: Frontiers Media S.A. 2021-10-01
Series:Frontiers in Molecular Neuroscience
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Online Access:https://www.frontiersin.org/articles/10.3389/fnmol.2021.721749/full