title: 3-Nitropropionic acid increases frataxin expression in human lymphoblasts and in transgenic rat PC12 cells.
abstract: Friedreich ataxia (FRDA) is the most common recessive ataxia caused by reduced expression of frataxin, a nuclear encoded mitochondrial protein. In this study we examined the effects of 3-nitropropionic acid (3-NP) on frataxin expression in FRDA patient and control lymphoblasts and in rat pheochromocytoma cell line (PC12) overexpressing human frataxin. Our studies showed an up-regulation of frataxin expression in both FRDA and control lymphoblasts following exposure to 3-NP. In addition, in transgenic frataxin overexpressing cells 3-NP caused an increase of frataxin protein.
authors.affiliation: BioGeM Consortium, c/o Dipartimento di Biologia e Patologia Cellulare e Molecolare, Università 'Federico II', Via S. Pansini, 5, 80131 Naples, Italy. turano@biogem.it
authors.forename: Mimmo
authors.initials: M
authors.lastname: Turano
authors.forename: Angela
authors.initials: A
authors.lastname: Tammaro
authors.forename: Irene
authors.initials: I
authors.lastname: De Biase
authors.forename: Maria Simona
authors.initials: MS
authors.lastname: Lo Casale
authors.forename: Giuseppina
authors.initials: G
authors.lastname: Ruggiero
authors.forename: Antonella
authors.initials: A
authors.lastname: Monticelli
authors.forename: Sergio
authors.initials: S
authors.lastname: Cocozza
authors.forename: Luigi
authors.initials: L
authors.lastname: Pianese
mesh_terms: D000818:Animals; D015153:Blotting, Western; D004305:Dose-Response Relationship, Drug; D004791:Enzyme Inhibitors / Q000494:pharmacology*; D005621:Friedreich Ataxia / Q000378:metabolism; D006801:Humans; D033862:Iron-Binding Proteins / Q000096:biosynthesis* / Q000187:drug effects* / Q000235:genetics; D008214:Lymphocytes / Q000187:drug effects*; D009574:Nitro Compounds; D018384:Oxidative Stress; D016716:PC12 Cells; D011422:Propionates / Q000494:pharmacology*; D051381:Rats; D013234:Stem Cells / Q000187:drug effects; D013997:Time Factors; D019076:Transgenes; D000096923:Frataxin
chemical_list: D004791:Enzyme Inhibitors; D033862:Iron-Binding Proteins; D009574:Nitro Compounds; D011422:Propionates; D000096923:Frataxin; C015392:3-nitropropionic acid