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.
3-Nitropropionic acid increases frataxin expression in humanlymphoblasts and in transgenic rat PC12 cells / Turano, Mimmo; Angela, Tammaro; Irene De, Biase; Maria Simona Lo, Casale; Ruggiero, Giuseppina; Antonella, Monticelli; Cocozza, Sergio; Luigi, Pianese. - In: NEUROSCIENCE LETTERS. - ISSN 0304-3940. - STAMPA. - 350:(2003), pp. 184-186.
3-Nitropropionic acid increases frataxin expression in humanlymphoblasts and in transgenic rat PC12 cells
TURANO, MIMMO;RUGGIERO, GIUSEPPINA;COCOZZA, SERGIO;
2003
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.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
