Expanded Polyglutamine Tract Itself Induces Cell Death in Cultured Cells

Kyoung Sook Bok; Hyangshuk Rhim; Young Do Yoo; Euiju Choi; Kwangseok Ahn; Ic Hwan Kim; Seongman Kang

Expanded Polyglutamine Tract Itself Induces Cell Death in Cultured Cells

Kyoung Sook Bok, Hyangshuk Rhim, Young Do Yoo, Euiju Choi, Kwangseok Ahn, Ic Hwan Kim, Seongman Kang

Research output: Contribution to journal › Article › peer-review

Abstract

Several neurodegenerative diseases including Huntington disease, Machado-Joseph disease and spinocerebellar ataxias type 1 are caused by expansion of a polyglutamine tract within their respective gene products. In order to assess the role of the tract, 293T cells were transfected with plasmids that contain various lengths of CAG repeat encoding polyglutamine without the repeat disorder proteins: (CAG)₂₇, (CAG)₄₀, (CAG)₈₀, (CAG)₁₃₀, and (CAG)₁₈₀. Except for (CAG)₂₇, and (CAG)₄₀, 293T cells showed a common set of morphological alterations such as shrinkage, rounding and surface blebbing when the expanded stretch was expressed. In addition, nuclear staining experiments showed chromatin condensation in COS-7 cells transfected with the vectors containing expanded CAG repeats. These results indicate that expanded polyglutamine itself is able to induce cell death, suggesting existence of a common molecular mechanism in the etiology of neurodegenerative polyglutamine diseases.

Original language	English
Pages (from-to)	398-402
Number of pages	5
Journal	Molecules and cells
Volume	9
Issue number	4
Publication status	Published - 1999 Aug 31

Keywords

Cell Death
Expansion
Polyglutamine

ASJC Scopus subject areas

Molecular Biology
Cell Biology

Cite this

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title = "Expanded Polyglutamine Tract Itself Induces Cell Death in Cultured Cells",

abstract = "Several neurodegenerative diseases including Huntington disease, Machado-Joseph disease and spinocerebellar ataxias type 1 are caused by expansion of a polyglutamine tract within their respective gene products. In order to assess the role of the tract, 293T cells were transfected with plasmids that contain various lengths of CAG repeat encoding polyglutamine without the repeat disorder proteins: (CAG)27, (CAG)40, (CAG)80, (CAG)130, and (CAG)180. Except for (CAG)27, and (CAG)40, 293T cells showed a common set of morphological alterations such as shrinkage, rounding and surface blebbing when the expanded stretch was expressed. In addition, nuclear staining experiments showed chromatin condensation in COS-7 cells transfected with the vectors containing expanded CAG repeats. These results indicate that expanded polyglutamine itself is able to induce cell death, suggesting existence of a common molecular mechanism in the etiology of neurodegenerative polyglutamine diseases.",

keywords = "Cell Death, Expansion, Polyglutamine",

author = "Bok, {Kyoung Sook} and Hyangshuk Rhim and Yoo, {Young Do} and Euiju Choi and Kwangseok Ahn and Kim, {Ic Hwan} and Seongman Kang",

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language = "English",

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AU - Bok, Kyoung Sook

AU - Rhim, Hyangshuk

AU - Yoo, Young Do

AU - Choi, Euiju

AU - Ahn, Kwangseok

AU - Kim, Ic Hwan

AU - Kang, Seongman

PY - 1999/8/31

Y1 - 1999/8/31

N2 - Several neurodegenerative diseases including Huntington disease, Machado-Joseph disease and spinocerebellar ataxias type 1 are caused by expansion of a polyglutamine tract within their respective gene products. In order to assess the role of the tract, 293T cells were transfected with plasmids that contain various lengths of CAG repeat encoding polyglutamine without the repeat disorder proteins: (CAG)27, (CAG)40, (CAG)80, (CAG)130, and (CAG)180. Except for (CAG)27, and (CAG)40, 293T cells showed a common set of morphological alterations such as shrinkage, rounding and surface blebbing when the expanded stretch was expressed. In addition, nuclear staining experiments showed chromatin condensation in COS-7 cells transfected with the vectors containing expanded CAG repeats. These results indicate that expanded polyglutamine itself is able to induce cell death, suggesting existence of a common molecular mechanism in the etiology of neurodegenerative polyglutamine diseases.

AB - Several neurodegenerative diseases including Huntington disease, Machado-Joseph disease and spinocerebellar ataxias type 1 are caused by expansion of a polyglutamine tract within their respective gene products. In order to assess the role of the tract, 293T cells were transfected with plasmids that contain various lengths of CAG repeat encoding polyglutamine without the repeat disorder proteins: (CAG)27, (CAG)40, (CAG)80, (CAG)130, and (CAG)180. Except for (CAG)27, and (CAG)40, 293T cells showed a common set of morphological alterations such as shrinkage, rounding and surface blebbing when the expanded stretch was expressed. In addition, nuclear staining experiments showed chromatin condensation in COS-7 cells transfected with the vectors containing expanded CAG repeats. These results indicate that expanded polyglutamine itself is able to induce cell death, suggesting existence of a common molecular mechanism in the etiology of neurodegenerative polyglutamine diseases.

KW - Cell Death

KW - Expansion

KW - Polyglutamine

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ER -

Expanded Polyglutamine Tract Itself Induces Cell Death in Cultured Cells

Abstract

Keywords

ASJC Scopus subject areas

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