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Misfolded SOD1 Associated with Motor Neuron Mitochondria Alters Mitochondrial Shape and Distribution Prior to Clinical Onset

Mutations in superoxide dismutase (SOD1) are causative for inherited amyotrophic lateral sclerosis. A proportion of SOD1 mutant protein is misfolded onto the cytoplasmic face of mitochondria in one or more spinal cord cell types. By construction of mice in which mitochondrially targeted enhanced gre...

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Detalles Bibliográficos
Autores principales: Vande Velde, Christine, McDonald, Karli K., Boukhedimi, Yasmin, McAlonis-Downes, Melissa, Lobsiger, Christian S., Bel Hadj, Samar, Zandona, Andre, Julien, Jean-Pierre, Shah, Sameer B., Cleveland, Don W.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2011
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3136936/
https://www.ncbi.nlm.nih.gov/pubmed/21779368
http://dx.doi.org/10.1371/journal.pone.0022031