LplA-mediated rescue of lethal myopathy and cardiomyopathy in mitochondrial lipoylation-deficient mammalian caused by Lipt1
Inherited mitochondrial metabolic disorders impair cellular energy generation and often lead to fatal metabolic crises. Mitochondrial lipoylation defects represent a severe case of such disorders that can lead to lethality but lacks effective treatment so far. In this study, we generated a viable mouse model with muscle-specific deficiency of the lipoyltransferase coded by Lipt1 to provide an in…
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