A characteristic of polyglutamine diseases is the increased propensity of disease proteins to aggregate, which is thought to be a major contributing factor to the underlying neurodegeneration. Healthy cells contain mechanisms for handling protein damage, the protein quality control, which must be impaired or inefficient to permit proteotoxicity under pathological conditions.
Cellular protein quality control and the evolution of aggregates in spinocerebellar ataxia type 3 (SCA3) / Seidel, K; Meister, M; Dugbartey, G. J; Zijlstra, M. P; Vinet, Jonathan; Brunt, E. R. P; van Leeuwen, F. W; Rüb, U; Kampinga, H. H; den Dunnen, W. F. A.. - In: NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY. - ISSN 0305-1846. - 38:(2012), pp. 548-558. [10.1111/j.1365-2990.2011.01220.x]
Cellular protein quality control and the evolution of aggregates in spinocerebellar ataxia type 3 (SCA3)
VINET, JONATHAN;
2012
Abstract
A characteristic of polyglutamine diseases is the increased propensity of disease proteins to aggregate, which is thought to be a major contributing factor to the underlying neurodegeneration. Healthy cells contain mechanisms for handling protein damage, the protein quality control, which must be impaired or inefficient to permit proteotoxicity under pathological conditions.
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