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Description

Ammonia directly inhibits critical TCA cycle enzymes, including KGDH, isocitrate dehydrogenase, and malate dehydrogenase, compromising oxidative phosphorylation (OXPHOS) (Prasad et al

Loss of CLPP alleviates mitochondrial cardiomyopathy without affecting the mammalian UPRmt

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This review aims to serve as a comprehensive reference for clinical and laboratory findings for those who are confronted with pediatric and adult patients with a possible diagnosis of a mitochondrial lcFAOD