University of Groningen
Type VII collagen in the intraocular environment
Wullink, Bart
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):
Wullink, B. (2019). Type VII collagen in the intraocular environment. Rijksuniversiteit Groningen.
Copyright
Other than for strictly personal use, it is not permitted to download or to forward/distribute the text or part of it without the consent of the author(s) and/or copyright holder(s), unless the work is under an open content license (like Creative Commons).
Take-down policy
If you believe that this document breaches copyright please contact us providing details, and we will remove access to the work immediately and investigate your claim.
Downloaded from the University of Groningen/UMCG research database (Pure): http://www.rug.nl/research/portal. For technical reasons the number of authors shown on this cover page is limited to 10 maximum.
COLOPHON
Layout and cover design: Design Your Thesis, www.designyourthesis.com Printing: Ridderprint B.V., www.ridderprint.nl
ISBN: 978-94-6375-414-9 Printed on recycled paper
Cover: Type VII collagen molecules, schematically depicted as hooked shapes in varying thickness, are dispersed (but arranged) to form an eye.
The printing of this thesis was financially supported by the the Prof. Mulder Stichting, the Graduate School of Medical Sciences, the Kolff Institute, the University of Groningen, Stichting Blindenhulp, Rotterdamse Stichting Blindenbelangen.
Copyright © 2019 by Bart Wullink. All rights reserved. Any unauthorized reprint or use of this material is prohibited. No part of this thesis may be reproduced, stored or transmitted in any form or by any means, without written permission of the author or, when appropriate, of the publishers of the publications.
Type VII collagen in the
intraocular environment
Proefschrift
ter verkrijging van de graad van doctor aan de Rijksuniversiteit Groningen
op gezag van de
rector magnificus prof. dr. E. Sterken en volgens besluit van het College voor Promoties.
De openbare verdediging zal plaatsvinden op maandag 8 juli 2019 om 9.00 uur
door
Bart Wullink
geboren op 2 oktober 1980 te Ermelo
Promotor
Prof. dr. J.M.M. HooymansCopromotores
Dr. L.I. Los Dr. T.G. van Kooten Dr. H.H. PasBeoordelingscommissie
Prof. dr. R.A. Bank Prof. dr. M.J. Jager Prof. dr. S.M. Imhof
ABBREVIATIONS
3H triple helix collagen domain
aa amino acid
ACD anterior chamber depth AEC 3-amino-9-ethylcarbazole AF anchoring fibril(s) BM(s) basement membrane(s) BSA bovine serum albumin
CA corpora amylacea
CD-31 endothelial cell marker COL7A1 gene encoding for Col VII Col VII type VII collagen D diopter
DAPI 2-(4-Amidinophenyl)-6-indolecarbamidine dihydrochloride DDEB dominant dystrophic epidermolysis bullosa
EB epidermolysis bullosa EBS epidermolysis bullosa simplex FITC fluorescein isothiocyanate FN3 fibronectin domain 3 GCL ganglion cell layer GFAP glial fibrillary acidic protein ILM inner limiting membrane IPL inner plexiform layer INL inner nuclear layer
iTEM immuno TEM
JEB junctional epidermolysis bullosa kDa kilodalton
LM light microscopy
mRNA messenger RNA
NC-1 non-collagenous domain 1 NFL nerve fiber layer
NPE non-pigmented epithelium (of ciliary body) OCT optical coherence tomography
OD oculus dexter (right eye) ONL outer nuclear layer OPL outer plexiform layer
Optos ultra-widefield scanning laser ophthalmoscope OS oculus sinister (left eye)
OU oculo uterique (both eyes) PAS periodic acid-Schiff PB(S) phosphate buffer(ed saline)
PE pigmented epithelium (of ciliary body) PKP penetrating keratoplasty
PR photoreceptors
RAMPO rabbit-anti-mouse peroxidase
RDEB recessive dystrophic epidermolysis bullosa RPE retinal pigmented epithelium
SARPO swineanti-rabbit peroxidase SMA smooth muscle actin Sup Info supporting information T8100 glycol methacrylate resin TBS Tris buffered saline
TEM transmission electron microscopy TRITC tetramethyl rhodamine isothiocyanate TTBS TBS containing 0.05% Tween-20
vb vitreous body
vWFA von Willebrand factor type A WOD Wet op Orgaandonatie
TABLE OF CONTENTS
Chapter 1 General introduction 9
Chapter 2 Type VII collagen expression in the human vitreoretinal
interface, corpora amylacea and inner retinal layers
31
Chapter 3 Type VII Collagen in the Human Accommodation System:
Expression in Ciliary Body, Zonules, and Lens Capsule
65
Chapter 4 Type VII Collagen in the Ocular Vasculature 93
Chapter 5 Recessive Dystrophic Epidermolysis Bullosa: type VII
collagen deficiency is not associated with intraocular defects
129
Chapter 6 General Discussion 159
Chapter 7 Summary 191
Chapter 8 Nederlandse samenvatting (Dutch summary) 197
Dankwoord (Acknowledgements) 203
