Progressive thalamocortical neuron loss in Cln5 deficient mice: Distinct effects in Finnish variant late infantile NCL

Progressive thalamocortical neuron loss in Cln5 deficient mice: Distinct effects in Finnish variant late infantile NCL

Finnish variant LINCL (vLINCLFin) is the result of mutations in the CLN5 gene. To gain insights into the pathological staging of this fatal pediatric disorder, we have undertaken a stereological analysis of the CNS of Cln5 deficient mice (Cln5−/−) at different stages of disease progression. Consiste...

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Bibliographic Details
Main Authors: Carina von Schantz, Catherine Kielar, Stine N. Hansen, Charlie C. Pontikis, Noreen A. Alexander, Outi Kopra, Anu Jalanko, Jonathan D. Cooper
Format: Article
Language:English
Published: Elsevier 2009-05-01
Series:Neurobiology of Disease
Subjects:
Finnish variant late infantile neuronal ceroid lipofuscinosis
Batten disease
CLN5
Thalamocortical neurodegeneration
Lysosomal storage disorder
Online Access:http://www.sciencedirect.com/science/article/pii/S096999610900028X

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http://www.sciencedirect.com/science/article/pii/S096999610900028X

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