Membrane trafficking and mitochondrial abnormalities precede subunit c deposition in a cerebellar cell model of juvenile neuronal ceroid lipofuscinosis

BACKGROUND: JNCL is a recessively inherited, childhood-onset neurodegenerative disease most-commonly caused by a ~1 kb CLN3 mutation. The resulting loss of battenin activity leads to deposition of mitochondrial ATP synthase, subunit c and a specific loss of CNS neurons. We previously generated Cln3(...

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Main Authors: Fossale, Elisa, Wolf, Pavlina, Espinola, Janice A, Lubicz-Nawrocka, Tanya, Teed, Allison M, Gao, Hanlin, Rigamonti, Dorotea, Cattaneo, Elena, MacDonald, Marcy E, Cotman, Susan L
Formato: Artigo
Idioma:Inglês
Publicado em: BioMed Central 2004
Assuntos:
Research Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC539297/
https://ncbi.nlm.nih.gov/pubmed/15588329
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/1471-2202-5-57
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