The yeast metacaspase is implicated in oxidative stress response in frataxin-deficient cells.

Abstract:

:Friedreich ataxia is the most common recessive neurodegenerative disease and is caused by reduced expression of mitochondrial frataxin. Frataxin depletion causes impairment in iron-sulfur cluster and heme biosynthesis, disruption of iron homeostasis and hypersensitivity to oxidants. Currently no pharmacological treatment blocks disease progression, although antioxidant therapies proved to benefit patients. We show that sensitivity of yeast frataxin-deficient cells to hydrogen peroxide is partially mediated by the metacaspase. Metacaspase deletion in frataxin-deficient cells results in recovery of antioxidant capacity and heme synthesis. In addition, our results suggest that metacaspase is associated with mitochondrial respiration, intracellular redox control and genomic stability.

journal_name

FEBS Lett

journal_title

FEBS letters

authors

Lefevre S,Sliwa D,Auchère F,Brossas C,Ruckenstuhl C,Boggetto N,Lesuisse E,Madeo F,Camadro JM,Santos R

doi

10.1016/j.febslet.2011.12.002

subject

Has Abstract

pub_date

2012-01-20 00:00:00

pages

143-8

issue

2

eissn

0014-5793

issn

1873-3468

pii

S0014-5793(11)00872-6

journal_volume

586

pub_type

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