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Approximately 7% of the nuclear exome encodes proteins targeted to mitochondria (10) including the majority of the subunits of the OXPHOS enzymes and dozens of proteins required for their coordinated assembly, together with proteins needed for mtDNA maintenance and gene expression (which together number into the hundreds—including numerous transcription and translation factors and ∼80 mitochondrial ribosomal proteins), mitochondrial import proteins, and enzymes involved in fatty acid oxidation, the Krebs cycle, and biosynthesis of lipids including steroid hormones, coenzyme Q 10 , heme, iron-sulfur clusters, and other cofactors

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Three-form amino acid matrix: Citrulline DL-Malate, Arginine HCl, and Arginine Alpha-Ketoglutarate

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