Takuya Norizuki1#, Taeko Sasaki1#, Yuji Suehiro2, Shohei Mitani2, Waka Kojima3,4, Koji Yamano3,4, Noriyuki Matsuda3, and Miyuki Sato1* (1. Laboratory of Molecular Membrane Biology, Institute for Molecular and Cellular Regulation, Gunma University 2. Department of Physiology, Tokyo Women's Medical University School of Medicine; 3. Department of Biomolecular Pathogenesis, Medical Research Laboratory, Institute of Integrated Research, Institute of Science Tokyo, 4. Intracellular Quality Control Project, Tokyo Metropolitan Institute of Medical Science)
About
Mitochondria possess their own DNA, and mitochondrial DNA is maternally inherited in most organisms. We previously showed that autophagic degradation of paternal mitochondria is responsible for this unique inheritance in the nematode Caenorhabditis elegans. However, the molecular mechanism underlying this process has remained unclear.
In this study, we show that BCAS3 is required for paternal mitochondrial degradation. We also found that loss of BCAS3 function impaired accumulation of machinery required for autophagosome formation around paternal mitochondria, suggesting that BCAS3 plays an important role in autophagosome formation. BCAS3 is also conserved in humans, and mutations in this gene have been reported to cause neurodevelopmental disorders. Our findings not only reveal the mechanism of paternal mitochondrial degradation but may also contribute to understanding the pathogenesis of neurodevelopmental disorders caused by BCAS3 mutations.
Paper information
Norizuki, T., Sasaki, T., Suehiro, Y., Mitani, S., Kojima, W., Yamano, K., Matsuda, N., and Sato, M. BCAS-3 is required for progression of autophagosome formation to degrade paternal mitochondria in Caenorhabditis elegans. iScience. 2026 29(7); 116345.
Online URL
https://www.cell.com/iscience/fulltext/S2589-0042(26)01720-7
