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Property "Has-20abstract" (as page type) with input value "The-20mitochondrial-20intramembrane-20rhomboid-20protease-20PARL-20has-20been-20implicated-20in-20spanerse-20functions-20-27-27in-20vitro-27-27,-20but-20its-20physiological-20role-20-27-27in-20vivo-27-27-20remains-20unclear.-20Here-20we-20show-20that-20-27-27Parl-27-27-20ablation-20in-20mouse-20causes-20a-20necrotizing-20encephalomyelopathy-20similar-20to-20Leigh-20syndrome,-20a-20mitochondrial-20disease-20characterized-20by-20disrupted-20energy-20production.-20Mice-20with-20conditional-20PARL-20deficiency-20in-20the-20nervous-20system,-20but-20not-20in-20muscle,-20develop-20a-20similar-20phenotype-20as-20germline-20-27-27Parl-27-27-20KOs,-20demonstrating-20the-20vital-20role-20of-20PARL-20in-20neurological-20homeostasis.-20Genetic-20modification-20of-20two-20major-20PARL-20substrates,-20PINK1-20and-20PGAM5,-20do-20not-20modify-20this-20severe-20neurological-20phenotype.-20-27-27Parl-27-27-3Csup-3E-2D-2F-2D-3C-2Fsup-3E-20brain-20mitochondria-20are-20affected-20by-20progressive-20ultrastructural-20changes-20and-20by-20defects-20in-20Complex-20III-20(CIII)-20activity,-20coenzyme-20Q-20(CoQ)-20biosynthesis,-20and-20mitochondrial-20calcium-20metabolism.-20PARL-20is-20necessary-20for-20the-20stable-20expression-20of-20TTC19,-20which-20is-20required-20for-20CIII-20activity,-20and-20of-20COQ4,-20which-20is-20essential-20in-20CoQ-20biosynthesis.-20Thus,-20PARL-20plays-20a-20previously-20overlooked-20constitutive-20role-20in-20the-20maintenance-20of-20the-20respiratory-20chain-20in-20the-20nervous-20system,-20and-20its-20deficiency-20causes-20progressive-20mitochondrial-20dysfunction-20and-20structural-20abnormalities-20leading-20to-20neuronal-20necrosis-20and-20Leigh-2Dlike-20syndrome." contains invalid characters or is incomplete and therefore can cause unexpected results during a query or annotation process.

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