Cln5-deficiency in mice leads to microglial activation, defective myelination and changes in lipid metabolism

Cln5-deficiency in mice leads to microglial activation, defective myelination and changes in lipid metabolism

CLN5 disease, late infantile variant phenotype neuronal ceroid lipofuscinosis, is a severe neurodegenerative disease caused by mutations in the CLN5 gene, which encodes a lysosomal protein of unknown function. Cln5-deficiency in mice leads to loss of thalamocortical neurons, and glial activation, bu...

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Bibliographic Details
Main Authors: Mia-Lisa Schmiedt, Tea Blom, Tomas Blom, Outi Kopra, Andrew Wong, Carina von Schantz-Fant, Elina Ikonen, Mervi Kuronen, Matti Jauhiainen, Jonathan D. Cooper, Anu Jalanko
Format: Article
Language:English
Published: Elsevier 2012-04-01
Series:Neurobiology of Disease
Subjects:
Neuronal ceroid lipofuscinoses
NCL
erCln5
Microglia
Lipid metabolism
Myelin
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996111003846

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http://www.sciencedirect.com/science/article/pii/S0969996111003846

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