Impaired flickering of the permeability transition pore causes SPG7 spastic paraplegia

BACKGROUND: Mutations of the mitochondrial protein paraplegin cause hereditary spastic paraplegia type 7 (SPG7), a so-far untreatable degenerative disease of the upper motoneuron with still undefined pathomechanism. The intermittent mitochondrial permeability transition pore (mPTP) opening, called f...

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Detalles Bibliográficos
Autores principales: Sambri, Irene, Massa, Filomena, Gullo, Francesca, Meneghini, Simone, Cassina, Laura, Carraro, Michela, Dina, Giorgia, Quattrini, Angelo, Patanella, Lorenzo, Carissimo, Annamaria, Iuliano, Antonella, Santorelli, Filippo, Codazzi, Franca, Grohovaz, Fabio, Bernardi, Paolo, Becchetti, Andrea, Casari, Giorgio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2020
Materias:
Research Paper
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7553352/
https://www.ncbi.nlm.nih.gov/pubmed/33045469
http://dx.doi.org/10.1016/j.ebiom.2020.103050

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