3-Nitropropionic acid increases frataxin expression in human lymphoblasts and in transgenic rat PC12 cells

Authors :: Luigi Pianese
Maria Simona Lo Casale
Angela Tammaro
Mimmo Turano
Irene De Biase
Sergio Cocozza
Antonella Monticelli
Giuseppina Ruggiero
Turano, Mimmo
Tammaro, A
DE BIASE, I
LO CASALE, M
Ruggiero, Giuseppina
Monticelli, A
Cocozza, Sergio
Pianese, L.
Source :: Neuroscience letters, 350 (2003): 184–186., info:cnr-pdr/source/autori:Turano M., Tammaro A., De Biase I., Lo Casale M.S., Ruggiero G., MONTICELLI A., Cocozza S., Pianese L./titolo:3-Nitropropionic acid increases frataxin expression in human lymphoblasts and in transgenic rat PC12 cells./doi:/rivista:Neuroscience letters (Print)/anno:2003/pagina_da:184/pagina_a:186/intervallo_pagine:184–186/volume:350
Publication Year :: 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.

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