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A Drosophila model for Duchenne muscular dystrophy Plas, M.C. van der

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A Drosophila model for Duchenne muscular dystrophy

Plas, M.C. van der

Citation

Plas, M. C. van der. (2008, January 24). A Drosophila model for Duchenne muscular dystrophy. Retrieved from https://hdl.handle.net/1887/12577

Version: Corrected Publisher’s Version

License: Licence agreement concerning inclusion of doctoral thesis in the Institutional Repository of the University of Leiden

Downloaded from: https://hdl.handle.net/1887/12577

Note: To cite this publication please use the final published version (if applicable).

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A Drosophila Model for Duchenne Muscular Dystrophy

The Role of Dystrophin in Muscle and Nervous System

Mariska C. van der Plas

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A Drosophila Model for Duchenne Muscular Dystrophy

Proefschrift

ter verkrijging van

de graad van Doctor aan de Universiteit Leiden,

op gezag van de Rector Magnificus prof. mr. P. F. van der Heijden, volgens besluit van het College voor Promoties

te verdedigen op donderdag 24 januari 2008 klokke 16:15 uur

door

Mariska C. van der Plas geboren te Gouda

in 1980

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Promotiecommissie

Promotor: Prof. Dr. J.N. Noordermeer Co-promotor: Dr. L.G. Fradkin

Referent: Dr. R.A. Baines, University of Manchester, UK

Overige leden: Prof. Dr. H.J. Tanke

Prof. Dr. G.J.B. van Ommen Prof. Dr. E.R. de Kloet Dr. J.J. Plomp

The studies described in this thesis were performed at the department of Molecular Cell Biology, Leiden University Medical Center.

This research has been made possible by a pionier grant of the Netherlands Organisation for Scientific Research (NWO) grant number 900-02-003. The financial support of Leica Microsystems B.V. in printing this thesis is gratefully acknowledged.

Cover: ‘Degenerating leaves’. Photographer: Walmink.

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“It was the best of times, it was the worst of times, it was the age of wisdom, it was the age of foolishness, it was the epoch of belief, it was the epoch of incredulity, it was the season of Light, it was the season of Darkness, it was the spring of hope, it was the winter of despair, we had everything before us, we had nothing before us, we were all going direct to heaven, we were all going direct the other way - in short, the period was so far like the present period, that some of its noisiest authorities insisted on its being received, for good or for evil, in the superlative degree of comparison only.”

Charles Dickens (1812-1870), A Tale of Two Cities.

“Where so many hours have been spent in convincing myself that I am right, is there not some reason to fear I may be wrong?”

Jane Austen (1775 - 1817), Sense and Sensibility.

“W..e a..p..o..l..o..g..i..z..e for the i..n..c..o..n..v..e..n..i..e..n..c..e”

(God’s Final Message to His Creation) Douglas Adams (1952-2001), So long, and thanks for all the fish.

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Contents of this thesis

Chapter 1 Introduction

9

Chapter 2 Embryonic expression patterns of the Drosophila

dystrophin-associated glycoprotein complex orthologs. 45 Dekkers LC, van der Plas MC, van Loenen PB, den Dunnen JT, van

Ommen GJ, Fradkin LG, Noordermeer JN.

Gene Expression Patterns 4(2) (2004) 153-159.

Chapter 3 Dystrophin Is Required for Appropriate Retrograde Control of Neurotransmitter Release at the Drosophila Neuromuscular Junction.

55

van der Plas MC, Pilgram GSK, Plomp JJ, de Jong A, Fradkin LG, Noordermeer JN.

The Journal of Neuroscience 26(1) (2006) 333–344.

Chapter 4 The Dp186 Dystrophin Isoform Mediates Regulation of Neurotransmitter Release at Central Synapses in Drosophila.

83

Fradkin LG, Baines RA, van der Plas MC, Noordermeer JN.

The Journal of Neuroscience, in revision.

Chapter 5 The Drosophila Dystrophin Dp117 Isoform Is Required For

Muscle Integrity. 101

van der Plas MC, Pilgram GSK, de Jong A, Bansraj MRKS, Fradkin LG, Noordermeer JN.

Mechanisms of Development 124 (2007) 617-630.

Chapter 6 The role of Drosophila Dystrobrevin in survival, muscle

integrity, and wing formation. 125

Van der Plas MC, Kremer A, de Jong AWM, Fradkin LG, Noordermeer JN.

Chapter 7 General Discussion 145

Chapter 8 Summary 159

Nederlandse samenvatting 163

List of publications 165

Curriculum vitae 167

Appendix Color figures 169

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