Targeting lipid peroxidation and mitochondrial imbalance in Friedreich's ataxia.

doi:10.1016/j.phrs.2015.05.015

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Article

Targeting lipid peroxidation and mitochondrial imbalance in Friedreich's ataxia.

Pharmacological research - 1 Sept 2015

Abeti Rosella, Uzun Ebru, Renganathan Indhushri, Honda Tadashi, Pook Mark A, Giunti Paola

Abstract excerpt

Friedreich's ataxia (FRDA) is an autosomal recessive disorder, caused by reduced levels of the protein frataxin. This protein is located in the mitochondria, where it functions in the biogenesis of iron-sulphur clusters (ISCs), which are important for the function of the mitochondrial respiratory chain complexes. Moreover, disruption in iron biogenesis may lead to oxidative stress. Oxidative stress can be the...

Topics

Animals
Antioxidants
Cell Death
Cells, Cultured
Disease Models, Animal
Fibroblasts
Friedreich Ataxia
Iron
Iron-Binding Proteins
Lipid Peroxidation
Membrane Potential, Mitochondrial

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Mice

Mice, Inbred C57BL

Mitochondria

Oxidative Stress

Phenanthrenes

Phenotype

Frataxin

Source

Open in PubMed

Identifiers

PMID: 26141703
DOI: 10.1016/j.phrs.2015.05.015

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