Exacerbated neuronal ceroid lipofuscinosis phenotype in Cln1/5 double-knockout mice

Both CLN1 and CLN5 deficiencies lead to severe neurodegenerative diseases of childhood, known as neuronal ceroid lipofuscinoses (NCLs). The broadly similar phenotypes of NCL mouse models, and the potential for interactions between NCL proteins, raise the possibility of shared or convergent disease m...

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Main Authors: Blom, Tea, Schmiedt, Mia-Lisa, Wong, Andrew M., Kyttälä, Aija, Soronen, Jarkko, Jauhiainen, Matti, Tyynelä, Jaana, Cooper, Jonathan D., Jalanko, Anu
Format: Artigo
Language:Inglês
Published: The Company of Biologists Limited 2013
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Research Article
Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC3597017/
https://ncbi.nlm.nih.gov/pubmed/23065637
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1242/dmm.010140
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