Article
TTC19 Plays a Husbandry Role on UQCRFS1 Turnover in the Biogenesis of Mitochondrial Respiratory Complex III.
Molecular cell - 6 Jul 2017
Bottani Emanuela, Cerutti Raffaele, Harbour Michael E, Ravaglia Sabrina, Dogan Sukru Anil, Giordano Carla, Fearnley Ian M, D'Amati Giulia, Viscomi Carlo, Fernandez-Vizarra Erika, Zeviani Massimo
Abstract excerpt
Loss-of-function mutations in TTC19 (tetra-tricopeptide repeat domain 19) have been associated with severe neurological phenotypes and mitochondrial respiratory chain complex III deficiency. We previously demonstrated the mitochondrial localization of TTC19 and its link with complex III biogenesis. Here we provide detailed insight into the mechanistic role of TTC19, by investigating a Ttc19?/? mouse model that...
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