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

Finnish variant LINCL (vLINCL(Fin)) 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. Cons...

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Bibliographische Detailangaben
Hauptverfasser: von Schantz, Carina, Kielar, Catherine, Hansen, Stine N, Pontikis, Charlie C, Alexander, Noreen A, Kopra, Outi, Jalanko, Anu, Cooper, Jonathan D
Format: Artigo
Sprache:Inglês
Veröffentlicht: 2009
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Article
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC2704904/
https://ncbi.nlm.nih.gov/pubmed/19385065
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.nbd.2009.02.001
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