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 | |
| State | Published - 2015 |
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Keywords
- Mitochondria
- Mitochondrial diseases
- Mouse models
- Oxidative phosphorylation
- OXPHOS
ASJC Scopus subject areas
- Cell Biology
- Molecular Biology
- Molecular Medicine
Cite this
Mitochondrial Diseases Part III : Therapeutic interventions in mouse models of OXPHOS deficiencies. / Peralta, Susana; Torraco, Alessandra; Iommarini, Luisa; Diaz, Francisca.
In: Mitochondrion, Vol. 23, 2015, p. 71-80.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Mitochondrial Diseases Part III
T2 - Therapeutic interventions in mouse models of OXPHOS deficiencies
AU - Peralta, Susana
AU - Torraco, Alessandra
AU - Iommarini, Luisa
AU - Diaz, Francisca
PY - 2015
Y1 - 2015
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 - Oxidative phosphorylation
KW - OXPHOS
UR - http://www.scopus.com/inward/record.url?scp=84937634662&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84937634662&partnerID=8YFLogxK
U2 - 10.1016/j.mito.2015.01.007
DO - 10.1016/j.mito.2015.01.007
M3 - Article
VL - 23
SP - 71
EP - 80
JO - Mitochondrion
JF - Mitochondrion
SN - 1567-7249
ER -