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Movements Speech disorder Cognition: Impaired Course: Progressive Laboratory Brain CT: Calcification in bilateral globus pallidus, thalamus & cerebellum Serum calcium & phosphate levels: Normal Also see Leigh syndrome(s) Cardiomyopathy Mitochondrial syndromes: Childhood Onset MC4DN11: Ataxia, Pyramidal syndrome & Cytochrome oxidase deficiency 151 FAM36A (Cytochrome c oxidase 20

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dsRNA foci were quantified using the particle analyzer function of Fiji

The tricarboxylic acid (TCA) cycle breaks down the carbon substrate of acetyl-CoA, derived from pyruvate, amino acids, and fatty acids, generating carbon dioxide and reducing NAD + to NADH and FAD 2+ to FADH 2 (21) (see Figure 1 )

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