Mitochondrial free radical overproduction due to respiratory chain impairment in the brain of a mouse model of Rett syndrome: protective effect of CNF1

Rett syndrome (RTT) is a pervasive neurodevelopmental disorder mainly caused by mutations in the X-linked MECP2 gene associated with severe intellectual disability, movement disorders, and autistic-like behaviors. Its pathogenesis remains mostly not understood and no effective therapy is available....

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Veröffentlicht in:Free radical biology & medicine 2015-06, Vol.83, p.167-177
Hauptverfasser: De Filippis, Bianca, Valenti, Daniela, de Bari, Lidia, De Rasmo, Domenico, Musto, Mattia, Fabbri, Alessia, Ricceri, Laura, Fiorentini, Carla, Laviola, Giovanni, Vacca, Rosa Anna
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container_title Free radical biology & medicine
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creator De Filippis, Bianca
Valenti, Daniela
de Bari, Lidia
De Rasmo, Domenico
Musto, Mattia
Fabbri, Alessia
Ricceri, Laura
Fiorentini, Carla
Laviola, Giovanni
Vacca, Rosa Anna
description Rett syndrome (RTT) is a pervasive neurodevelopmental disorder mainly caused by mutations in the X-linked MECP2 gene associated with severe intellectual disability, movement disorders, and autistic-like behaviors. Its pathogenesis remains mostly not understood and no effective therapy is available. High circulating levels of oxidative stress markers in patients and the occurrence of oxidative brain damage in MeCP2-deficient mouse models suggest the involvement of oxidative stress in RTT pathogenesis. However, the molecular mechanism and the origin of the oxidative stress have not been elucidated. Here we demonstrate that a redox imbalance arises from aberrant mitochondrial functionality in the brain of MeCP2-308 heterozygous female mice, a condition that more closely recapitulates that of RTT patients. The marked increase in the rate of hydrogen peroxide generation in the brain of RTT mice seems mainly produced by the dysfunctional complex II of the mitochondrial respiratory chain. In addition, both membrane potential generation and mitochondrial ATP synthesis are decreased in RTT mouse brains when succinate, the complex II respiratory substrate, is used as an energy source. Respiratory chain impairment is brain area specific, owing to a decrease in either cAMP-dependent phosphorylation or protein levels of specific complex subunits. Further, we investigated whether the treatment of RTT mice with the bacterial protein CNF1, previously reported to ameliorate the neurobehavioral phenotype and brain bioenergetic markers in an RTT mouse model, exerts specific effects on brain mitochondrial function and consequently on hydrogen peroxide production. In RTT brains treated with CNF1, we observed the reactivation of respiratory chain complexes, the rescue of mitochondrial functionality, and the prevention of brain hydrogen peroxide overproduction. These results provide definitive evidence of mitochondrial reactive oxygen species overproduction in RTT mouse brain and highlight CNF1 efficacy in counteracting RTT-related mitochondrial defects. •The origin of oxidative stress in Rett syndrome (RTT) was studied.•Impaired mitochondrial complex II induces H2O2 overproduction in RTT mouse brain.•Reactivation of mitochondrial respiratory chain by CNF1 prevented H2O2 production.•Drugs targeting brain mitochondria functionality could be considered for RTT therapy.
doi_str_mv 10.1016/j.freeradbiomed.2015.02.014
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control</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>De Filippis, Bianca</creatorcontrib><creatorcontrib>Valenti, Daniela</creatorcontrib><creatorcontrib>de Bari, Lidia</creatorcontrib><creatorcontrib>De Rasmo, Domenico</creatorcontrib><creatorcontrib>Musto, Mattia</creatorcontrib><creatorcontrib>Fabbri, Alessia</creatorcontrib><creatorcontrib>Ricceri, Laura</creatorcontrib><creatorcontrib>Fiorentini, Carla</creatorcontrib><creatorcontrib>Laviola, Giovanni</creatorcontrib><creatorcontrib>Vacca, Rosa Anna</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Free radical biology &amp; medicine</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>De Filippis, Bianca</au><au>Valenti, Daniela</au><au>de Bari, Lidia</au><au>De Rasmo, Domenico</au><au>Musto, Mattia</au><au>Fabbri, Alessia</au><au>Ricceri, Laura</au><au>Fiorentini, Carla</au><au>Laviola, Giovanni</au><au>Vacca, Rosa Anna</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Mitochondrial free radical overproduction due to respiratory chain impairment in the brain of a mouse model of Rett syndrome: protective effect of CNF1</atitle><jtitle>Free radical biology &amp; medicine</jtitle><addtitle>Free Radic Biol Med</addtitle><date>2015-06</date><risdate>2015</risdate><volume>83</volume><spage>167</spage><epage>177</epage><pages>167-177</pages><issn>0891-5849</issn><eissn>1873-4596</eissn><abstract>Rett syndrome (RTT) is a pervasive neurodevelopmental disorder mainly caused by mutations in the X-linked MECP2 gene associated with severe intellectual disability, movement disorders, and autistic-like behaviors. Its pathogenesis remains mostly not understood and no effective therapy is available. High circulating levels of oxidative stress markers in patients and the occurrence of oxidative brain damage in MeCP2-deficient mouse models suggest the involvement of oxidative stress in RTT pathogenesis. However, the molecular mechanism and the origin of the oxidative stress have not been elucidated. Here we demonstrate that a redox imbalance arises from aberrant mitochondrial functionality in the brain of MeCP2-308 heterozygous female mice, a condition that more closely recapitulates that of RTT patients. The marked increase in the rate of hydrogen peroxide generation in the brain of RTT mice seems mainly produced by the dysfunctional complex II of the mitochondrial respiratory chain. In addition, both membrane potential generation and mitochondrial ATP synthesis are decreased in RTT mouse brains when succinate, the complex II respiratory substrate, is used as an energy source. Respiratory chain impairment is brain area specific, owing to a decrease in either cAMP-dependent phosphorylation or protein levels of specific complex subunits. Further, we investigated whether the treatment of RTT mice with the bacterial protein CNF1, previously reported to ameliorate the neurobehavioral phenotype and brain bioenergetic markers in an RTT mouse model, exerts specific effects on brain mitochondrial function and consequently on hydrogen peroxide production. In RTT brains treated with CNF1, we observed the reactivation of respiratory chain complexes, the rescue of mitochondrial functionality, and the prevention of brain hydrogen peroxide overproduction. These results provide definitive evidence of mitochondrial reactive oxygen species overproduction in RTT mouse brain and highlight CNF1 efficacy in counteracting RTT-related mitochondrial defects. •The origin of oxidative stress in Rett syndrome (RTT) was studied.•Impaired mitochondrial complex II induces H2O2 overproduction in RTT mouse brain.•Reactivation of mitochondrial respiratory chain by CNF1 prevented H2O2 production.•Drugs targeting brain mitochondria functionality could be considered for RTT therapy.</abstract><cop>United States</cop><pub>Elsevier Inc</pub><pmid>25708779</pmid><doi>10.1016/j.freeradbiomed.2015.02.014</doi><tpages>11</tpages><orcidid>https://orcid.org/0000-0003-2438-6449</orcidid></addata></record>
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subjects Adenosine Triphosphate - metabolism
Animals
Bacterial Toxins - administration & dosage
Bacterial Toxins - metabolism
Brain - drug effects
Brain - metabolism
Brain - pathology
Disease Models, Animal
Electron Transport
Electron Transport Complex II - metabolism
Energy metabolism
Escherichia coli Proteins - administration & dosage
Escherichia coli Proteins - metabolism
Female
Free radicals
Free Radicals - metabolism
Humans
Immunoblotting
Male
Methyl-CpG-Binding Protein 2 - physiology
Mice
Mice, Inbred C57BL
Mice, Knockout
Mitochondria - metabolism
Mitochondria - pathology
Mitochondrial dysfunction
Mitochondrial Membranes - metabolism
Mutation - genetics
Oxidation-Reduction
Oxidative Stress
Phenotype
Reactive oxygen species
Reactive Oxygen Species - metabolism
Rett syndrome
Rett Syndrome - etiology
Rett Syndrome - metabolism
Rett Syndrome - pathology
Rett Syndrome - prevention & control
title Mitochondrial free radical overproduction due to respiratory chain impairment in the brain of a mouse model of Rett syndrome: protective effect of CNF1
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