A two-step mitochondrial import pathway couples the disulfide relay with matrix complex I biogenesis

Authors: Esra Peker, Konstantin Weiss, Jiyao Song, Christine Zarges, Sarah Gerlich, Volker Boehm, Aleksandra Trifunovic, Thomas Langer, Niels H. Gehring, Thomas Becker, and Jan Riemer

Journal of Cell Biology, May 09 2023

In this study, scientists characterized the complex maturation pathway of the complex I assembly factor for protein import in mitochondria. To help explore the contribution of NDUFAF8 redox state and normal levels on NDUFAF8 knockout cells, the team performed a proliferation assay on the Omni live-cell imager, finding that the addition of NDUFAF8 to knock out cells restored growth of these cells in galactose-containing medium. Overall, the researchers concluded that the “two-step import pathway might serve as a mechanism to sense and integrate the activity of the disulfide relay in the IMS with matrix processes, and thereby constitute an additional regulatory layer that allows the tight coordination of assembly of complexes from proteins of dual genetic origin.”