Disruption of the autophagy-lysosome pathway is involved in neuropathology of the nclf mouse model of neuronal ceroid lipofuscinosis.

Disruption of the autophagy-lysosome pathway is involved in neuropathology of the nclf mouse model of neuronal ceroid lipofuscinosis.

Variant late-infantile neuronal ceroid lipofuscinosis, a fatal lysosomal storage disorder accompanied by regional atrophy and pronounced neuron loss in the brain, is caused by mutations in the CLN6 gene. CLN6 is a non-glycosylated endoplasmic reticulum (ER)-resident membrane protein of unknown funct...

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Bibliographic Details
Main Authors: Melanie Thelen, Markus Damme, Michaela Schweizer, Christian Hagel, Andrew M S Wong, Jonathan D Cooper, Thomas Braulke, Giovanna Galliciotti
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2012-01-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC3335005?pdf=render

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http://europepmc.org/articles/PMC3335005?pdf=render

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