Mitochondrial Function in Hereditary Spastic Paraplegia: Deficits in SPG7 but Not SPAST Patient-Derived Stem Cells

Mutations in SPG7 and SPAST are common causes of hereditary spastic paraplegia (HSP). While some SPG7 mutations cause paraplegin deficiency, other SPG7 mutations cause increased paraplegin expression. Mitochondrial function has been studied in models that are paraplegin-deficient (human, mouse, and...

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Bibliographic Details
Main Authors: Gautam Wali, Kishore Raj Kumar, Erandhi Liyanage, Ryan L. Davis, Alan Mackay-Sim, Carolyn M. Sue
Format: Article
Language:English
Published: Frontiers Media S.A. 2020-08-01
Series:Frontiers in Neuroscience
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Online Access:https://www.frontiersin.org/article/10.3389/fnins.2020.00820/full