Article
CHCHD2 accumulates in distressed mitochondria and facilitates oligomerization of CHCHD10.
Human molecular genetics - 15 Nov 2018
Huang Xiaoping, Wu Beverly P, Nguyen Diana, Liu Yi-Ting, Marani Melika, Hench Jürgen, Bénit Paule, Kozjak-Pavlovic Vera, Rustin Pierre, Frank Stephan, Narendra Derek P
Abstract excerpt
Mutations in paralogous mitochondrial proteins CHCHD2 and CHCHD10 cause autosomal dominant Parkinson Disease (PD) and Amyotrophic Lateral Sclerosis/Frontotemporal Dementia (ALS/FTD), respectively. Using newly generated CHCHD2, CHCHD10 and CHCHD2/10 double knockout cell lines, we find that the proteins are partially functionally redundant, similarly distributed throughout the mitochondrial cristae, and form...
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