Reduced cell size, chromosomal aberration and altered proliferation rates are characteristics and confounding factors in the STHdh cell model of Huntington disease

Abstract Huntington disease is a fatal neurodegenerative disorder caused by a CAG repeat expansion in the gene encoding the huntingtin protein. Expression of the mutant protein disrupts various intracellular pathways and impairs overall cell function. In particular striatal neurons seem to be most v...

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Autores principales:	Elisabeth Singer, Carolin Walter, Jonasz J. Weber, Ann-Christin Krahl, Ulrike A. Mau-Holzmann, Nadine Rischert, Olaf Riess, Laura E. Clemensson, Huu P. Nguyen
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2017
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/baca3f0edb594d1fb92d80a01ad67420
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spelling	oai:doaj.org-article:baca3f0edb594d1fb92d80a01ad674202021-12-02T15:05:10ZReduced cell size, chromosomal aberration and altered proliferation rates are characteristics and confounding factors in the STHdh cell model of Huntington disease10.1038/s41598-017-17275-42045-2322https://doaj.org/article/baca3f0edb594d1fb92d80a01ad674202017-12-01T00:00:00Zhttps://doi.org/10.1038/s41598-017-17275-4https://doaj.org/toc/2045-2322Abstract Huntington disease is a fatal neurodegenerative disorder caused by a CAG repeat expansion in the gene encoding the huntingtin protein. Expression of the mutant protein disrupts various intracellular pathways and impairs overall cell function. In particular striatal neurons seem to be most vulnerable to mutant huntingtin-related changes. A well-known and commonly used model to study molecular aspects of Huntington disease are the striatum-derived STHdh cell lines generated from wild type and huntingtin knock-in mouse embryos. However, obvious morphological differences between wild type and mutant cell lines exist, which have rarely been described and might not have always been considered when designing experiments or interpreting results. Here, we demonstrate that STHdh cell lines display differences in cell size, proliferation rate and chromosomal content. While the chromosomal divergence is considered to be a result of the cells’ tumour characteristics, differences in size and proliferation, however, were confirmed in a second non-immortalized Huntington disease cell model. Importantly, our results further suggest that the reported phenotypes can confound other study outcomes and lead to false conclusions. Thus, careful experimental design and data analysis are advised when using these cell models.Elisabeth SingerCarolin WalterJonasz J. WeberAnn-Christin KrahlUlrike A. Mau-HolzmannNadine RischertOlaf RiessLaura E. ClemenssonHuu P. NguyenNature PortfolioarticleMedicineRScienceQENScientific Reports, Vol 7, Iss 1, Pp 1-13 (2017)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Elisabeth Singer Carolin Walter Jonasz J. Weber Ann-Christin Krahl Ulrike A. Mau-Holzmann Nadine Rischert Olaf Riess Laura E. Clemensson Huu P. Nguyen Reduced cell size, chromosomal aberration and altered proliferation rates are characteristics and confounding factors in the STHdh cell model of Huntington disease
description	Abstract Huntington disease is a fatal neurodegenerative disorder caused by a CAG repeat expansion in the gene encoding the huntingtin protein. Expression of the mutant protein disrupts various intracellular pathways and impairs overall cell function. In particular striatal neurons seem to be most vulnerable to mutant huntingtin-related changes. A well-known and commonly used model to study molecular aspects of Huntington disease are the striatum-derived STHdh cell lines generated from wild type and huntingtin knock-in mouse embryos. However, obvious morphological differences between wild type and mutant cell lines exist, which have rarely been described and might not have always been considered when designing experiments or interpreting results. Here, we demonstrate that STHdh cell lines display differences in cell size, proliferation rate and chromosomal content. While the chromosomal divergence is considered to be a result of the cells’ tumour characteristics, differences in size and proliferation, however, were confirmed in a second non-immortalized Huntington disease cell model. Importantly, our results further suggest that the reported phenotypes can confound other study outcomes and lead to false conclusions. Thus, careful experimental design and data analysis are advised when using these cell models.
format	article
author	Elisabeth Singer Carolin Walter Jonasz J. Weber Ann-Christin Krahl Ulrike A. Mau-Holzmann Nadine Rischert Olaf Riess Laura E. Clemensson Huu P. Nguyen
author_facet	Elisabeth Singer Carolin Walter Jonasz J. Weber Ann-Christin Krahl Ulrike A. Mau-Holzmann Nadine Rischert Olaf Riess Laura E. Clemensson Huu P. Nguyen
author_sort	Elisabeth Singer
title	Reduced cell size, chromosomal aberration and altered proliferation rates are characteristics and confounding factors in the STHdh cell model of Huntington disease
title_short	Reduced cell size, chromosomal aberration and altered proliferation rates are characteristics and confounding factors in the STHdh cell model of Huntington disease
title_full	Reduced cell size, chromosomal aberration and altered proliferation rates are characteristics and confounding factors in the STHdh cell model of Huntington disease
title_fullStr	Reduced cell size, chromosomal aberration and altered proliferation rates are characteristics and confounding factors in the STHdh cell model of Huntington disease
title_full_unstemmed	Reduced cell size, chromosomal aberration and altered proliferation rates are characteristics and confounding factors in the STHdh cell model of Huntington disease
title_sort	reduced cell size, chromosomal aberration and altered proliferation rates are characteristics and confounding factors in the sthdh cell model of huntington disease
publisher	Nature Portfolio
publishDate	2017
url	https://doaj.org/article/baca3f0edb594d1fb92d80a01ad67420
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Reduced cell size, chromosomal aberration and altered proliferation rates are characteristics and confounding factors in the STHdh cell model of Huntington disease

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