4′‐Phosphopantetheine corrects CoA, iron, and dopamine metabolic defects in mammalian models of PKAN - Jeong, Suh Young; Hogarth, Penelope; Placzek, Andrew; Gregory, Allison M; Fox, Rachel; Zhen, Dolly; Hamada, Jeffrey; van der Zwaag, Marianne; Lambrechts, Roald; Jin, Haihong; Nilsen, Aaron; Cobb, Jared; et al. (EMBO molecular medicine, 2019)
Keywords: 4′‐phosphopantetheine; Acyl carrier protein; Animals; Basal ganglia; Biomarkers; Biomarkers - metabolism; Brain research; Central nervous system diseases; coenzyme A; Coenzyme A - metabolism; Defects; Dopamine; Dopamine - metabolism; Drug development; Dystonia; EMBO08; EMBO27; Gene expression; Genotype; Homeostasis; Iron; Iron - metabolism; Kinases; Mammals; Metabolism; Mice; Mitochondria; Movement disorders; Mutation; NBIA; Neurodegeneration; Oxidative phosphorylation; PANK2; Pantetheine - analogs & derivatives; Pantetheine - pharmacology; Pantetheine - therapeutic use; Pantothenate kinase; Pantothenate Kinase-Associated Neurodegeneration - drug therapy; Pantothenate Kinase-Associated Neurodegeneration - metabolism; Pathogenesis; Pharmacodynamics; Phosphorylation; Phosphotransferases (Alcohol Group Acceptor) - metabolism; PKAN; Pyruvic acid; Sulfur; NBIA; PANK2; 4′‐phosphopantetheine; PKAN; coenzyme A; 4′-phosphopantetheine