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3-Nitropropionic acid increases frataxin expression in human lymphoblasts and in transgenic rat PC12 cells
Mimmo Turano1, Angela Tammaro, Irene De Biase
1BioGeM Consortium, c/o Dipartimento di Biologia e Patologia Cellulare e Molecolare, Università 'Federico II', Via S. Pansini, 5, 80131 Naples, Italy. turano@biogem.it
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.