Citation
Voer, G. de. (2008, May 7). The cln-3 genes of Caenorhabditis elegans : making C. elegans models for Juvenile Neuronal Ceroid Lipofuscinosis. Retrieved from
https://hdl.handle.net/1887/12840
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The cln-3 genes of Caenorhabditis elegans
Making C. elegans models for Juvenile Neuronal Ceroid Lipofuscinosis
The cln-3 genes of Caenorhabditis elegans
Making C. elegans models for Juvenile Neuronal Ceroid Lipofuscinosis
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 donderdag 24 januari 2008
klokke 13:45 uur door
Gert de Voer
geboren te Almelo in 1973
The printing of this thesis is financially supported by De Jurriaanse Stichting and Genzyme Europe B.V.
“People demand freedom of speech to make up for the freedom of thought which they avoid”
S O R E N A A B Y E K I E R K E G A A R D (1813-1855)
I N D E X 7
Making C. elegans models for Juvenile Neuronal Ceroid Lipofuscinosis
The cln-3 genes of Caenorhabditis elegans
CHAPTER 1 Introduction...9 Juvenile Neuronal Ceroid Lipofuscinosis
Lysosomes
Lysosomal storage diseases
The Neuronal Ceroid Lipofuscinoses JNCL disease models
Caenorhabditis elegans
Aim and outline of this thesis
CHAPTER 2 Caenorhabditis elegans as a model for lysosomal storage disorders...63 De Voer G, Peters DJM, Taschner PEM
Manuscript submitted
CHAPTER 3 Caenorhabditis elegans homologues of the CLN3 gene,
mutated in Juvenile Neuronal Ceroid Lipofuscinsosis...95 De Voer G, Jansen G, van Ommen GJB, Peters DJM, Taschner PEM Eur J Paediatr Neurol. 2001;5 Suppl A:115-20
CHAPTER 4 Deletion of the Caenorhabditis elegans homologues of the CLN3 gene, involved in human Juvenile Neuronal Ceroid
Lipofuscinosis (JNCL), causes a mild progeric phenotype...107 De Voer G, van der Bent P, Rodrigues AJ, van Ommen GJB, Peters DJ, Taschner PE
J Inherit Metab Dis. 2005;28(6):1065-80
CHAPTER 5 Overexpression of Subunit c, the main component of the storage material in Juvenile Neuronal Ceroid Lipofuscinosis (JNCL), causes disruption of mitochondria in C. elegans and subsequent death ...133 De Voer G, de Keizer ROB, van der Bent P, van Ommen GJB, Peters DJM, Taschner PEM
Manuscript submitted
CHAPTER 6 Discussion...159
CHAPTER 7 Summary...169 Nederlandse samenvatting
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