Phenotypic characterization of a mouse model of juvenile neuronal ceroid lipofuscinosis

Phenotypic characterization of a mouse model of juvenile neuronal ceroid lipofuscinosis

Juvenile neuronal ceroid lipofuscinosis (JNCL) is an autosomal recessively inherited neurodegenerative disorder that results from mutations in the CLN3 gene. JNCL is characterized by accumulation of autofluorescent lysosomal storage bodies, vision loss, seizures, progressive cognitive and motor decl...

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Bibliographic Details
Main Authors: Martin L. Katz, Gary S. Johnson, Gregory E. Tullis, Bo Lei
Format: Article
Language:English
Published: Elsevier 2008-02-01
Series:Neurobiology of Disease
Subjects:
Behavior
Neurodegeneration
Mouse model
Lysosomal storage
Retina
Brain
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996107002021

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http://www.sciencedirect.com/science/article/pii/S0969996107002021

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