Sulforaphane improves mitochondrial metabolism in fibroblasts from patients with fragile X-associated tremor and ataxia syndrome - Napoli, Eleonora; Flores, Amanda; Mansuri, Yasmeen; Hagerman, Randi J.; Giulivi, Cecilia (Neurobiology of disease, 2021)
Keywords: Aged; Aged, 80 and over; Antioxidants; Ataxia - metabolism; Bioenergetics; Brain; Energy Metabolism - drug effects; Female; Fibroblasts; Fibroblasts - drug effects; Fibroblasts - metabolism; Fragile X Syndrome - metabolism; Humans; In Vitro Techniques; Iron - metabolism; Isothiocyanates - pharmacology; Male; Middle Aged; Mitochondria - drug effects; Mitochondria - metabolism; Neurodegeneration; NF-E2-Related Factor 2 - drug effects; NF-E2-Related Factor 2 - metabolism; NRF2; Oxidative Stress - drug effects; Phytochemicals; Proteasome Endopeptidase Complex - drug effects; Proteasome Endopeptidase Complex - metabolism; Sulfoxides - pharmacology; Tremor - metabolism; Triplet nucleotide repeat diseases; Unfolded Protein Response; Antioxidants; Brain; Triplet nucleotide repeat diseases; Phytochemicals; Bioenergetics; NRF2; Neurodegeneration; Fibroblasts; Unfolded protein response