Article
Depletion of WFS1 compromises mitochondrial function in hiPSC-derived neuronal models of Wolfram syndrome.
Stem cell reports - 9 May 2023
Zatyka Malgorzata, Rosenstock Tatiana R, Sun Congxin, Palhegyi Adina M, Hughes Georgina W, Lara-Reyna Samuel, Astuti Dewi, di Maio Alessandro, Sciauvaud Axel, Korsgen Miriam E, Stanulovic Vesna, Kocak Gamze, Rak Malgorzata, Pourtoy-Brasselet Sandra, Winter Katherine, Varga Thiago, Jarrige Margot, Polvèche Hélène, Correia Joao, Frickel Eva-Maria, Hoogenkamp Maarten, Ward Douglas G, Aubry Laetitia, Barrett Timothy, Sarkar Sovan
Abstract excerpt
Mitochondrial dysfunction involving mitochondria-associated ER membrane (MAM) dysregulation is implicated in the pathogenesis of late-onset neurodegenerative diseases, but understanding is limited for rare early-onset conditions. Loss of the MAM-resident protein WFS1 causes Wolfram syndrome (WS), a rare early-onset neurodegenerative disease that has been linked to mitochondrial abnormalities. Here we demonstrate...
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