Novel interactions of CLN5 support molecular networking between Neuronal Ceroid Lipofuscinosis proteins.

BMC Cell Biology
Annina LylyAija Kyttälä

Abstract

Neuronal ceroid lipofuscinoses (NCLs) comprise at least eight genetically characterized neurodegenerative disorders of childhood. Despite of genetic heterogeneity, the high similarity of clinical symptoms and pathology of different NCL disorders suggest cooperation between different NCL proteins and common mechanisms of pathogenesis. Here, we have studied molecular interactions between NCL proteins, concentrating specifically on the interactions of CLN5, the protein underlying the Finnish variant late infantile form of NCL (vLINCLFin). We found that CLN5 interacts with several other NCL proteins namely, CLN1/PPT1, CLN2/TPP1, CLN3, CLN6 and CLN8. Furthermore, analysis of the intracellular targeting of CLN5 together with the interacting NCL proteins revealed that over-expression of PPT1 can facilitate the lysosomal transport of mutated CLN5FinMajor, normally residing in the ER and in the Golgi complex. The significance of the novel interaction between CLN5 and PPT1 was further supported by the finding that CLN5 was also able to bind the F1-ATPase, earlier shown to interact with PPT1. We have described novel interactions between CLN5 and several NCL proteins, suggesting a modifying role for these proteins in the pathogenesis of in...Continue Reading

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Datasets Mentioned

BETA
GM130

Methods Mentioned

BETA
co-immunoprecipitation
pull-down
transfection
PCR
pull-downs
immunoprecipitation
pull down
protein folding
confocal microscopy
co-immunoprecipation

Software Mentioned

Adobe Illustrator
FinMajor
Adobe Photoshop
TCS NT

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