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Dysmyelination not demyelination causes neurological symptoms in preweaned mice in a murine model of Cockayne syndrome

Cockayne syndrome (CS) is a rare autosomal recessive neurodegenerative disease that is associated with mutations in either of two transcription-coupled DNA repair genes, CSA or CSB. Mice with a targeted mutation in the Csb gene (Cs-b(m/m)) exhibit a milder phenotype compared with human patients with...

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Detalhes bibliográficos
Main Authors: Revet, Ingrid, Feeney, Luzviminda, Tang, Amy A., Huang, Eric J., Cleaver, James E.
Formato: Artigo
Idioma:Inglês
Publicado em: National Academy of Sciences 2012
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3311387/
https://ncbi.nlm.nih.gov/pubmed/22393014
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.1202621109
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