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. BCAS3 is also conserved in humans, and mutations in this gene have been reported to cause neurodevelopmental disorder. 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.

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