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UvA-DARE is a service provided by the library of the University of Amsterdam (http://dare.uva.nl)

UvA-DARE (Digital Academic Repository)

Lipotoxicity in adrenoleukodystrophySize matters!van de Beek, M.-C.

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Citation for published version (APA):van de Beek, M-C. (2018). Lipotoxicity in adrenoleukodystrophy: Size matters!.

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Download date: 07 Jan 2020

UITNODIGING

voor het bijwonen van de openbare verdediging van het proefschrift

Lipotoxicity in adrenoleukodystrophySize matters!

op donderdag 11 januari 2018 om 12:00 in de Agnietenkapel. Oudezijdsvoorburgwal 231 te Amsterdam

Receptie aansluitend in de Agnietenkapel

Feest Donderdag 11 januari 2018 20:00 - 00:00 in V.O.C. Café de Schreierstoren Prins Hendrikkade 94 te Amsterdam

Malu-Clair Brejaart-van de BeekBalthasar Floriszstraat 7-21071TZ Amsterdammalu-clair@hotmail.com

ParanimfenMegan van de Beekmeganvandebeek@gmail.com

Noa van de Beeknoavdbeek@gmail.com

Lipotoxicity in adrenoleukodystrophySize matters!

Malu-Clair van de Beek

Lipotoxicity in

adrenoleuk

odystrophy | Malu-Clair van

de Beek

15017 - Malu-Clair van de Beek_OM.indd 1 27-11-17 16:20

Lipotoxicity in AdrenoleukodystrophySize matters!

Malu-Clair van de Beek

The work described in this thesis was carried out at the Laboratory Genetic Metabolic Diseases, Department of Clinical Chemistry, Academic Medical Center, University of Amsterdam, The Netherlands. The research was financially supported by grants from the European Leukodystrophy Association (ELA 2014-014-1).

Lipotoxicity in adrenoleukodystrophy Size matters!Malu-Clair van de BeekThesis, University of Amsterdam, AmsterdamISBN: 978-94-6299-757-8Cover design: Malu-Clair van de BeekLay-out design: Ridderprint, The NetherlandsPrinted by Ridderprint, The Netherlands

Copyright © Malu-Clair van de Beek, 2017All rights reserved. No part of this thesis may be reproduced, stored in a retrieval system or transmitted in any way of by any means without prior permission of the author.

Lipotoxicity in AdrenoleukodystrophySize matters!

ACADEMISCH PROEFSCHRIFT

ter verkrijging van de graad van doctor aan de Universiteit van Amsterdam op gezag van de Rector Magnificus

prof. dr. ir. K.I.J. Maex ten overstaan van een door het College voor Promoties ingestelde commissie,

in het openbaar te verdedigen in de Agnietenkapel op donderdag 11 januari 2018, te 12:00 uur

door Malu-Clair van de Beek geboren te Hilversum

Promotiecommissie

Promotores: Prof. dr. R.J.A. Wanders AMC – Universiteit van Amsterdam Prof. dr. F.A. Wijburg AMC – Universiteit van Amsterdam

Copromotores: Dr. S. Kemp AMC – Universiteit van Amsterdam Prof. dr. B.T. Poll-The AMC – Universiteit van Amsterdam

Overige leden: Prof. dr. C.E.M. Hollak AMC – Universiteit van Amsterdam Prof. dr. R.C.M. Hennekam AMC – Universiteit van Amsterdam Prof. dr. M. Baes Katholieke Universiteit Leuven Prof. dr. A.K. Groen AMC – Universiteit van Amsterdam Dr. F.M. Vaz AMC – Universiteit van Amsterdam Dr. G.E.M.Abbink Vrije Universiteit Amsterdam

Faculteit der Geneeskunde

Table of contents

Chapter 1 Introduction

Chapter 2 Method for measurement of peroxisomal beta-oxidation and de novo C26:0 synthesis activity in living cells using stable-isotope labeled docosanoic acid Peroxisomes: Methods and Protocols (2017) 45-54

Chapter 3 C26:0-carnitine is a new biomarker for adrenoleukodystrophy in mice and men PLoS One (2016) 11(4):e0154597

Chapter 4 Comparison of C26:0-carnitine and C26:0-lysophosphatidylcholine as diagnostic markers in dried bloodspots from newborns and patients with adrenoleukodystrophy Molecular Genetics and Metabolism (2017) doi:10.1016/j.ymgme.2017.10.012

Chapter 5 Lipid-induced endoplasmic reticulum stress in adrenoleukodystrophy Biochimica et Biophysica Acta (2017) 1863(9):2255-65

Chapter 6 Redirecting very long-chain fatty acid synthesis in adrenoleukodystrophy

Chapter 7 Summary, General discussion & Future research

Chapter 8 Nederlandse samenvatting

Appendix Curriculum vitae List of publications PhD portfolio Dankwoord

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154 156 157 160

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