Mitochondrial Diseases Part III: Therapeutic interventions in mouse models of OXPHOS deficiencies

Susana Peralta; Alessandra Torraco; Luisa Iommarini; Francisca Diaz

doi:10.1016/j.mito.2015.01.007

Mitochondrial Diseases Part III: Therapeutic interventions in mouse models of OXPHOS deficiencies

Susana Peralta, Alessandra Torraco, Luisa Iommarini, Francisca Diaz

Neurology

Research output: Contribution to journal › Review article › peer-review

9 Scopus citations

Abstract

Mitochondrial defects are the cause of numerous disorders affecting the oxidative phosphorylation system (OXPHOS) in humans leading predominantly to neurological and muscular degeneration. The molecular origin, manifestations, and progression of mitochondrial diseases have a broad spectrum, which makes very challenging to find a globally effective therapy. The study of the molecular mechanisms underlying the mitochondrial dysfunction indicates that there is a wide range of pathways, enzymes and molecules that can be potentially targeted for therapeutic purposes. Therefore, focusing on the pathology of the disease is essential to design new treatments. In this review, we will summarize and discuss the different therapeutic interventions tested in some mouse models of mitochondrial diseases emphasizing the molecular mechanisms of action and their potential applications.

Original language	English (US)
Pages (from-to)	71-80
Number of pages	10
Journal	Mitochondrion
Volume	23
DOIs	https://doi.org/10.1016/j.mito.2015.01.007
State	Published - Jul 24 2015

Keywords

Mitochondria
Mitochondrial diseases
Mouse models
OXPHOS
Oxidative phosphorylation

ASJC Scopus subject areas

Molecular Medicine
Molecular Biology
Cell Biology

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10.1016/j.mito.2015.01.007

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title = "Mitochondrial Diseases Part III: Therapeutic interventions in mouse models of OXPHOS deficiencies",

abstract = "Mitochondrial defects are the cause of numerous disorders affecting the oxidative phosphorylation system (OXPHOS) in humans leading predominantly to neurological and muscular degeneration. The molecular origin, manifestations, and progression of mitochondrial diseases have a broad spectrum, which makes very challenging to find a globally effective therapy. The study of the molecular mechanisms underlying the mitochondrial dysfunction indicates that there is a wide range of pathways, enzymes and molecules that can be potentially targeted for therapeutic purposes. Therefore, focusing on the pathology of the disease is essential to design new treatments. In this review, we will summarize and discuss the different therapeutic interventions tested in some mouse models of mitochondrial diseases emphasizing the molecular mechanisms of action and their potential applications.",

keywords = "Mitochondria, Mitochondrial diseases, Mouse models, OXPHOS, Oxidative phosphorylation",

author = "Susana Peralta and Alessandra Torraco and Luisa Iommarini and Francisca Diaz",

note = "Funding Information: We would like to express our gratitude to Jose Oca-Cossio for critical comments of this manuscript. FD research has been funded by the James and Esther King Research Program Florida Department of Health (grant number 08KN-01 ), by the National Institute of Health (grant number GM101225 ) and by the United Mitochondrial Disease Foundation (grant number 14050R ). Publisher Copyright: {\textcopyright} 2015 Elsevier B.V.",

year = "2015",

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doi = "10.1016/j.mito.2015.01.007",

language = "English (US)",

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T2 - Therapeutic interventions in mouse models of OXPHOS deficiencies

AU - Peralta, Susana

AU - Torraco, Alessandra

AU - Iommarini, Luisa

AU - Diaz, Francisca

N1 - Funding Information: We would like to express our gratitude to Jose Oca-Cossio for critical comments of this manuscript. FD research has been funded by the James and Esther King Research Program Florida Department of Health (grant number 08KN-01 ), by the National Institute of Health (grant number GM101225 ) and by the United Mitochondrial Disease Foundation (grant number 14050R ). Publisher Copyright: © 2015 Elsevier B.V.

PY - 2015/7/24

Y1 - 2015/7/24

N2 - Mitochondrial defects are the cause of numerous disorders affecting the oxidative phosphorylation system (OXPHOS) in humans leading predominantly to neurological and muscular degeneration. The molecular origin, manifestations, and progression of mitochondrial diseases have a broad spectrum, which makes very challenging to find a globally effective therapy. The study of the molecular mechanisms underlying the mitochondrial dysfunction indicates that there is a wide range of pathways, enzymes and molecules that can be potentially targeted for therapeutic purposes. Therefore, focusing on the pathology of the disease is essential to design new treatments. In this review, we will summarize and discuss the different therapeutic interventions tested in some mouse models of mitochondrial diseases emphasizing the molecular mechanisms of action and their potential applications.

AB - Mitochondrial defects are the cause of numerous disorders affecting the oxidative phosphorylation system (OXPHOS) in humans leading predominantly to neurological and muscular degeneration. The molecular origin, manifestations, and progression of mitochondrial diseases have a broad spectrum, which makes very challenging to find a globally effective therapy. The study of the molecular mechanisms underlying the mitochondrial dysfunction indicates that there is a wide range of pathways, enzymes and molecules that can be potentially targeted for therapeutic purposes. Therefore, focusing on the pathology of the disease is essential to design new treatments. In this review, we will summarize and discuss the different therapeutic interventions tested in some mouse models of mitochondrial diseases emphasizing the molecular mechanisms of action and their potential applications.

KW - Mitochondria

KW - Mitochondrial diseases

KW - Mouse models

KW - OXPHOS

KW - Oxidative phosphorylation

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JF - Mitochondrion

SN - 1567-7249

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Mitochondrial Diseases Part III: Therapeutic interventions in mouse models of OXPHOS deficiencies

Abstract

Keywords

ASJC Scopus subject areas

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