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

ABSTRACT:

Background

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).

Results

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

SUBMITTER: Lyly A 

PROVIDER: S-EPMC2790443 | biostudies-literature | 2009 Nov

REPOSITORIES: biostudies-literature