Abstract

Friedreich ataxia (FRDA) is a common form of ataxia caused by decreased expression of the mitochondrial protein frataxin. Oxidative damage of mitochondria is thought to play a key role in the pathogenesis of the disease. Therefore, a possible therapeutic strategy should be directed to an antioxidant protection against mitochondrial damage. Indeed, treatment of FRDA patients with the antioxidant idebenone has been shown to improve neurological functions. The yeast frataxin knock-out model of the disease shows mitochondrial iron accumulation, iron-sulfur cluster defects and high sensitivity to oxidative stress. By flow cytometry analysis we studied reactive oxygen species (ROS) production of yeast frataxin mutant cells treated with two antioxidants, N-acetyl-L-cysteine and a mitochondrially-targeted analog of vitamin E, confirming that mitochondria are the main site of ROS production in this model. Furthermore we found a significant reduction of ROS production and a decrease in the mitochondrial mass in mutant cells treated with rapamycin, an inhibitor of TOR kinases, most likely due to autophagy of damaged mitochondria.

Autore Pugliese

• Marobbio Carlo Marya , Pisano Isabella , Palmieri Luigi , Porcelli Vito

Tutti gli autori

• MAROBBIO C.M.;PISANO I.;PALMIERI L.;PORCELLI V.

Titolo volume/Rivista

Non Disponibile

Anno di pubblicazione

2012

ISSN

1567-7249

ISBN

Non Disponibile

Numero di citazioni Wos

21

Ultimo Aggiornamento Citazioni

Non Disponibile

Numero di citazioni Scopus

23

Ultimo Aggiornamento Citazioni

Non Disponibile

Settori ERC

Non Disponibile

Codici ASJC

Non Disponibile