In vivo detection of oxidized proteins: a practical approach to tissue-derived mitochondria.

Francesca Maltecca, Giorgio Casari

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

Mitochondria are the major producers of free radical oxygen species (ROS) as well as the major target of oxidative damage. Defects in the mitochondrial respiratory chain complexes can increase ROS production and reduce ROS removal, leading to oxidative modification of proteins, lipids, and DNA. AAA proteases of the inner mitochondrial membrane, paraplegin and AFG3L2, participate in the biogenesis and maintenance of respiratory chain complexes. These proteins form hetero-oligomeric paraplegin/AFG3L2 and homo-oligomeric AFG3L2 complexes named m-AAA proteases. Inactivation of m-AAA proteases causes respiratory defects and altered mitochondrial morphology both in yeast and in mammals. In fact, mouse models defective for Afg3l2 display a very severe neurological syndrome and die within two weeks after birth. They display widespread morphological alterations of mitochondria in the central and peripheral nervous system and deficiencies in respiratory chain complex I and in complex III, which are major producers of ROS in physiological and especially in pathological conditions. Therefore, an efficient and reliable methodology to monitor the effect of increased ROS production is useful for accurately phenotyping cellular and animal models mutants in m-AAA. By measuring carbonyl formation as marker of protein oxidation, we have shown that respiratory defects cause oxidative damage in Afg3l2 mutants, indicating that oxidative stress is crucial in the pathogenesis of m-AAA deficiency.

Original languageEnglish
Pages (from-to)257-267
Number of pages11
JournalMethods in molecular biology (Clifton, N.J.)
Volume648
Publication statusPublished - 2010

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Reactive Oxygen Species
Mitochondria
Electron Transport
Proteins
Electron Transport Complex I
Electron Transport Complex III
Peripheral Nervous System
Mitochondrial Membranes
Free Radicals
Mammals
Oxidative Stress
Peptide Hydrolases
Central Nervous System
Animal Models
Yeasts
Maintenance
Parturition
Oxygen
Lipids
DNA

ASJC Scopus subject areas

  • Medicine(all)

Cite this

In vivo detection of oxidized proteins : a practical approach to tissue-derived mitochondria. / Maltecca, Francesca; Casari, Giorgio.

In: Methods in molecular biology (Clifton, N.J.), Vol. 648, 2010, p. 257-267.

Research output: Contribution to journalArticle

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