Impaired flickering of the permeability transition pore causes SPG7 spastic paraplegia

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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Bibliographic Details
Main Authors: 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: Article
Language:English
Published: Elsevier 2020-11-01
Series:EBioMedicine
Subjects:
Hereditary spastic paraplegia
Paraplegin
SPG7
Mitochondria
Permeability transition pore
Synaptic vesicles
Online Access:http://www.sciencedirect.com/science/article/pii/S2352396420304266

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http://www.sciencedirect.com/science/article/pii/S2352396420304266

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