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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 fl...

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Bibliografiske detaljer
Principais autores: Irene Sambri, Filomena Massa, Francesca Gullo, Simone Meneghini, Laura Cassina, Michela Carraro, Giorgia Dina, Angelo Quattrini, Lorenzo Patanella, Annamaria Carissimo, Antonella Iuliano, Filippo Santorelli, Franca Codazzi, Fabio Grohovaz, Paolo Bernardi, Andrea Becchetti, Giorgio Casari
Format: Artigo
Sprog:Inglês
Udgivet: Elsevier 2020-11-01
Serier:EBioMedicine
Fag:
Online adgang:http://www.sciencedirect.com/science/article/pii/S2352396420304266
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