Gute Nachbarschaft unter Mitochondrien
Impaired complex IV activity in response to loss of LRPPRC function can be compensated by mitochondrial hyperfusion
Stéphane G. Rolland, Elisa Motori, Nadin Memar, Jürgen Hench, Stephan Frank, Konstanze F. Winklhofer, andBarbara Conradt
Mitochondria, the powerhouses of the cell, constantly change their shape by fusing and dividing. How these two opposite processes are controlled remains unclear. In our study, we identified the Caenorhabditis elegans homolog of the human mitochondrial protein LRPPRC (leucine-rich pentatricopeptide repeat containing), which has been previously associated with the neurodegenerative French Canadian Leigh Syndrome. Analysis of this protein revealed an evolutionary conserved pathway that regulates mitochondrial shape. Specifically, we show that mitochondria transiently form a highly connected network to compensate for a decrease of the activity of the complex IV of the electron transport chain.