Progressive Polyglutamine Repeat Expansion in Peripheral Blood Cells and Sperm of Transgenic Huntington’s Disease Monkeys

The expanded CAG repeat results in somatic mosaicism and genetic anticipation in Huntington’s disease (HD). Here we report a longitudinal study examining CAG repeat instability in lymphocytes and sperm of three HD monkeys throughout their whole life-span that encompass the prodromal to symptomatic s...

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Autores principales: Clever, Faye, Cho, In Ki, Yang, Jingjing, Chan, Anthony W.S.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: IOS Press 2019
Materias:
Research Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6839466/
https://www.ncbi.nlm.nih.gov/pubmed/31561381
http://dx.doi.org/10.3233/JHD-190359
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author Clever, Faye
Cho, In Ki
Yang, Jingjing
Chan, Anthony W.S.
author_facet Clever, Faye
Cho, In Ki
Yang, Jingjing
Chan, Anthony W.S.
author_sort Clever, Faye
collection PubMed
description The expanded CAG repeat results in somatic mosaicism and genetic anticipation in Huntington’s disease (HD). Here we report a longitudinal study examining CAG repeat instability in lymphocytes and sperm of three HD monkeys throughout their whole life-span that encompass the prodromal to symptomatic stages of HD. We demonstrate a progressive increase in CAG repeat length in lymphocytes and sperm as the animals aged. We also examined the impact of CAG repeat length on expansion rate, which showed a clear linear correlation up to 62Q, and high instability after. Our findings stress the importance of further investigation in CAG instability in peripheral blood cells longitudinally.
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spelling pubmed-68394662019-11-20 Progressive Polyglutamine Repeat Expansion in Peripheral Blood Cells and Sperm of Transgenic Huntington’s Disease Monkeys Clever, Faye Cho, In Ki Yang, Jingjing Chan, Anthony W.S. J Huntingtons Dis Research Report The expanded CAG repeat results in somatic mosaicism and genetic anticipation in Huntington’s disease (HD). Here we report a longitudinal study examining CAG repeat instability in lymphocytes and sperm of three HD monkeys throughout their whole life-span that encompass the prodromal to symptomatic stages of HD. We demonstrate a progressive increase in CAG repeat length in lymphocytes and sperm as the animals aged. We also examined the impact of CAG repeat length on expansion rate, which showed a clear linear correlation up to 62Q, and high instability after. Our findings stress the importance of further investigation in CAG instability in peripheral blood cells longitudinally. IOS Press 2019-10-24 /pmc/articles/PMC6839466/ /pubmed/31561381 http://dx.doi.org/10.3233/JHD-190359 Text en © 2019 – IOS Press and the authors. All rights reserved https://creativecommons.org/licenses/by-nc/4.0/ This is an open access article distributed under the terms of the Creative Commons Attribution Non-Commercial (CC BY-NC 4.0) License (https://creativecommons.org/licenses/by-nc/4.0/) , which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Research Report
Clever, Faye
Cho, In Ki
Yang, Jingjing
Chan, Anthony W.S.
Progressive Polyglutamine Repeat Expansion in Peripheral Blood Cells and Sperm of Transgenic Huntington’s Disease Monkeys
title Progressive Polyglutamine Repeat Expansion in Peripheral Blood Cells and Sperm of Transgenic Huntington’s Disease Monkeys
title_full Progressive Polyglutamine Repeat Expansion in Peripheral Blood Cells and Sperm of Transgenic Huntington’s Disease Monkeys
title_fullStr Progressive Polyglutamine Repeat Expansion in Peripheral Blood Cells and Sperm of Transgenic Huntington’s Disease Monkeys
title_full_unstemmed Progressive Polyglutamine Repeat Expansion in Peripheral Blood Cells and Sperm of Transgenic Huntington’s Disease Monkeys
title_short Progressive Polyglutamine Repeat Expansion in Peripheral Blood Cells and Sperm of Transgenic Huntington’s Disease Monkeys
title_sort progressive polyglutamine repeat expansion in peripheral blood cells and sperm of transgenic huntington’s disease monkeys
topic Research Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6839466/
https://www.ncbi.nlm.nih.gov/pubmed/31561381
http://dx.doi.org/10.3233/JHD-190359
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