3-Nitropropionic acid increases frataxin expression in human lymphoblasts and in transgenic rat PC12 cells.
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.
Turano
Mimmo
M
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
Tammaro
Angela
A
De Biase
Irene
I
Lo Casale
Maria Simona
MS
Ruggiero
Giuseppina
G
Monticelli
Antonella
A
Cocozza
Sergio
S
Pianese
Luigi
L
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
D004791:Enzyme Inhibitors; D033862:Iron-Binding Proteins; D009574:Nitro Compounds; D011422:Propionates; D000096923:Frataxin; C015392:3-nitropropionic acid