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University of Groningen Cell-autonomous and non-cell autonomous protection of DNAJB6 in Huntington’s disease Bason, Matteo

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University of Groningen

Cell-autonomous and non-cell autonomous protection of DNAJB6 in Huntington’s disease Bason, Matteo

DOI:

10.33612/diss.101925993

IMPORTANT NOTE: You are advised to consult the publisher's version (publisher's PDF) if you wish to cite from it. Please check the document version below.

Document Version

Publisher's PDF, also known as Version of record

Publication date: 2019

Link to publication in University of Groningen/UMCG research database

Citation for published version (APA):

Bason, M. (2019). Cell-autonomous and non-cell autonomous protection of DNAJB6 in Huntington’s disease. University of Groningen. https://doi.org/10.33612/diss.101925993

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Cell-autonomous

and non-cell autonomous protection

of DNAJB6

in Huntington’s disease

Matteo Bason

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The research described in this thesis was conducted at the Department of Cell Biology, University Medical Center Groningen, University of Groningen, The Netherlands.

This research was supported by the Prinses Beatrix Foundation / de Nederlandse Hersenstichting (grant # F2012(1)-104).

Cover designed by Matteo Bason and Ilse Modder (artwork: ‘Study of Hands’, Albrecht Dürer, 1506)

Thesis printed by Gilderprint ISBN (print): 9789463238885 ISBN (electronic): 9789463238885

Copyright©2019 by Matteo Bason. All rights reserved. No part of this thesis may be reproduced, stored in a retrieval system, or transmitted in any form or by any means without permission of the author.

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Cell-autonomous and non-cell

autonomous protection of DNAJB6

in Huntington’s disease

PhD Thesis

to obtain the degree of PhD at the University of Groningen

on the authority of the

Rector Magnificus Prof. C. Wijmenga and in accordance with

the decision by the College of Deans.

This thesis will be defended in public on 2 December 2019 at 9:00 hours

by

Matteo Bason

born on 04 August 1983 in Brescia, Italy

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Supervisors:

Prof. H.H. Kampinga

Prof. H.W.G.M. Boddeke

Assessment Committee:

Prof. E.A.J. Reits

Prof. Y. Nagai

Prof. U.L.M. Eisel

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Paranymphs

Despina Serlidaki

Marco dal Lago

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Table of contents

Chapter 1: Outline of the Thesis

Page 9

Chapter 2: Introduction

Page 13

Chapter 3: A D.melanogaster model for the expression of

transgenes in neurons and astrocytes

Page 89

Chapter 4: Neuronal expression of the chaperone DNAJB6 results

in cell autonomous protection in Huntington’s disease

Page 105

Chapter 5: Astrocytic expression of the chaperone DNAJB6 results

in non-cell autonomous protection in Huntington’s disease

Page 125

Chapter 6: Discussion

Page 151

Samenvatting

Page 173

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