Abstract 2934 Comparative metabolomics identifies a shunt pathway for phenylalanine degradation in electron transport chain deficient mutants of the model organism C. elegans

D158N variant maintains metal binding but has significantly reduced phosphatase activity and fails to interact with BNIP3/ NIX via co-immunoprecipitation.Thus, we uncover a single point mutation in PPTC7 which perturbs both proposed functions of PPTC7 as a phosphatase and mitophagy suppressor.Overall, these findings are the first to highlight the key mechanisms of PPTC7-associated mitochondrial disease.