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Article

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

Neurobiology of disease - 1 May 2009

von Schantz Carina, Kielar Catherine, Hansen Stine N, Pontikis Charlie C, Alexander Noreen A, Kopra Outi, Jalanko Anu, Cooper Jonathan D

Abstract excerpt

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. Consistent with human vLINCL(Fin), these Cln5-/- mice displayed a relatively late onset regional atrophy and generalized...

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Identifiers

PMID: 19385065
DOI: 10.1016/j.nbd.2009.02.001
PMCID: PMC2704904

Genes

NCL
CLN5

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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