Citation
Pril, R. de. (2011, February 23). The ubiquitin proteasome system in Huntington disease : impairment of the proteolytic machinery aggravates huntingtin aggregation and toxicity. Retrieved from
https://hdl.handle.net/1887/16530
Version: Corrected Publisher’s Version
License: Licence agreement concerning inclusion of doctoral thesis in the Institutional Repository of the University of Leiden
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The Ubiquitin Proteasome System in Huntington disease
Impairment of the proteolytic machinery aggravates huntingtin aggregation and toxicity
Remko de Pril
The Ubiquitin Proteasome System in Huntington disease
Impairment of the proteolytic machinery aggravates huntingtin aggregation and toxicity
Proefschrift
ter verkrijging van
de graad van Doctor aan de Universiteit Leiden, op gezag van Rector Magnificus prof.mr. P.F. van der Heijden,
volgens besluit van het College voor Promoties te verdedigen op woensdag 23 februari 2011
klokke 16.15 uur
door Remko de Pril geboren te Woerden
in 1975
The research described in this thesis was financially supported by the “Prinses Beatrix Fonds”, MAR 99-0113
Table of contents
Chapter 1: Introduction 7
Chapter 2: Conformational diseases: An umbrella for various neurological disorders with an impaired ubiquitin-proteasome system
Neurobiology of Aging 27 (4): 515-523 31
Chapter 3: Accumulation of aberrant ubiquitin induces aggregate formation and cell death in polyglutamine diseases
Human Molecular Genetics 13 (16): 1803-1813 45 Chapter 4: Modest proteasomal inhibition by aberrant ubiquitin exacerbates
aggregate formation in a Huntington disease mouse model
Molecular and Cellular Neuroscience 43 (3): 281-286 65 Chapter 5: Ubiquitin-conjugating enzyme E2-25K increases aggregate
formation and cell death in polyglutamine diseases
Molecular and Cellular Neuroscience 34(1): 10-19 79
Chapter 6: Discussion 99
Abbreviations 111
References 113
Summary 133
Nederlandse Samenvatting 135
Curriculum Vitae 137
Publicaties 138
Dankwoord 139
