iPSC-derived neuronal models of PANK2-associated neurodegeneration reveal mitochondrial dysfunction contributing to early disease

Mutations in PANK2 lead to neurodegeneration with brain iron accumulation. PANK2 has a role in the biosynthesis of coenzyme A (CoA) from dietary vitamin B5, but the neuropathological mechanism and reasons for iron accumulation remain unknown. In this study, atypical patient-derived fibroblasts were...

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Detalles Bibliográficos
Autores principales: Arber, Charles, Angelova, Plamena R., Wiethoff, Sarah, Tsuchiya, Yugo, Mazzacuva, Francesca, Preza, Elisavet, Bhatia, Kailash P., Mills, Kevin, Gout, Ivan, Abramov, Andrey Y., Hardy, John, Duce, James A., Houlden, Henry, Wray, Selina
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2017
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5581181/
https://www.ncbi.nlm.nih.gov/pubmed/28863176
http://dx.doi.org/10.1371/journal.pone.0184104

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5581181/
https://www.ncbi.nlm.nih.gov/pubmed/28863176
http://dx.doi.org/10.1371/journal.pone.0184104

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