Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease
Volume 1822, Issue 2, February 2012, Pages 212-225
Changes in mitochondrial glutathione levels and protein thiol oxidation in ∆yfh1 yeast cells and the lymphoblasts of patients with Friedreich's ataxia
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Highlights
► Decrease in mitochondrial GSH/GSSG ratio in Δyfh1 yeast cells and the lymphoblasts of patients with FRDA. ► Increase in glutathione–protein adducts in both yeast and human frataxin-deficient cells. ► Thiol oxidation of key mitochondrial proteins, such as KGDH and subunits of respiratory chain complexes III and IV. ► Existence of a regulatory process involving both iron and glutathione in yeast and human cells.
Abbreviations
FRDA
Friedreich's ataxia
GSH
reduced glutathione
GSSG
oxidized disulfide glutathione
Grx
glutaredoxin
KGDH
α-ketoglutarate dehydrogenase
ISDH
isocitrate dehydrogenase
ROS
reactive oxygen species
YNB
yeast nitrogen base
Keywords
Friedreich's ataxia
Glutathione
Iron
Mitochondrion
Thiol oxidation
Protein glutathionylation
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