Distinct Early Molecular Responses to Mutations Causing vLINCL and JNCL Presage ATP Synthase Subunit C Accumulation in Cerebellar Cells

Variant late-infantile neuronal ceroid lipofuscinosis (vLINCL), caused by CLN6 mutation, and juvenile neuronal ceroid lipofuscinosis (JNCL), caused by CLN3 mutation, share clinical and pathological features, including lysosomal accumulation of mitochondrial ATP synthase subunit c, but the unrelated...

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Detalles Bibliográficos
Autores principales: Cao, Yi, Staropoli, John F., Biswas, Sunita, Espinola, Janice A., MacDonald, Marcy E., Lee, Jong-Min, Cotman, Susan L.
Formato: Texto
Lenguaje:English
Publicado: Public Library of Science 2011
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3040763/
https://www.ncbi.nlm.nih.gov/pubmed/21359198
http://dx.doi.org/10.1371/journal.pone.0017118

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