Literature DB >> 14550925

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

Mimmo Turano1, Angela Tammaro, Irene De Biase, Maria Simona Lo Casale, Giuseppina Ruggiero, Antonella Monticelli, Sergio Cocozza, Luigi Pianese.   

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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Year:  2003        PMID： 14550925     DOI： 10.1016/s0304-3940(03)00906-6

Source DB:  PubMed          Journal:  Neurosci Lett        ISSN： 0304-3940            Impact factor:   3.046


  5 in total

Review 1.  Friedreich ataxia-update on pathogenesis and possible therapies.

Authors:  Max Voncken; Panos Ioannou; Martin B Delatycki
Journal:  Neurogenetics       Date:  2003-12-19       Impact factor: 2.660

Review 2.  Small molecules affecting transcription in Friedreich ataxia.

Authors:  Joel M Gottesfeld
Journal:  Pharmacol Ther       Date:  2007-08-09       Impact factor: 12.310

3.  Recombinant human erythropoietin increases frataxin protein expression without increasing mRNA expression.

Authors:  Fabio Acquaviva; Imma Castaldo; Alessandro Filla; Manuela Giacchetti; Daniele Marmolino; Antonella Monticelli; Michele Pinelli; Francesco Saccà; Sergio Cocozza
Journal:  Cerebellum       Date:  2008       Impact factor: 3.847

4.  PPAR-gamma agonist Azelaoyl PAF increases frataxin protein and mRNA expression: new implications for the Friedreich's ataxia therapy.

Authors:  Daniele Marmolino; Fabio Acquaviva; Michele Pinelli; Antonella Monticelli; Imma Castaldo; Alessandro Filla; Sergio Cocozza
Journal:  Cerebellum       Date:  2008-12-23       Impact factor: 3.847

Review 5.  Intracellular iron transport and storage: from molecular mechanisms to health implications.

Authors:  Elizabeth L MacKenzie; Kenta Iwasaki; Yoshiaki Tsuji
Journal:  Antioxid Redox Signal       Date:  2008-06       Impact factor: 8.401
  5 in total

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