Part II: Mouse models of OXPHOS deficiencies caused by defects in regulatory factors and other components required for mitochondrial function

Part II: Mouse models of OXPHOS deficiencies caused by defects in regulatory factors and other components required for mitochondrial function. Luisa Iommarini, Susana Peralta, Alessandra Torraco, Francisca Diaz, P, Mitochondrion, Available online 29 January 2015, ISSN 1567-7249, http://dx.doi.org/10.1016/j.mito.2015.01.008.


Interestingly, in contrast to what has been observed extensively in clinical studies, the mice did not show any oxidative damage. This difference could be explained because in mice the total deletion of Fxn happens in a certain tissue at a specific time, whereas FRDA is characterized by partial Frataxin deficiency in all cells throughout the lifetime.

Mitochondrial diseases Part III: Therapeutic interventions in mouse models of oxphos deficiencies

Mitochondrial diseases Part III: Therapeutic interventions in mouse models of oxphos deficiencies. Mitochondrion, Available online 29 January 2015, ISSN 1567-7249, http://dx.doi.org/10.1016/j.mito.2015.01.007.

This review summarize and discuss the different therapeutic interventions tested in some mouse models of mitochondrial diseases laying emphasis on the molecular mechanisms of action and their potential applications.

Emerging critical roles of Fe-S clusters in DNA replication and repair

Emerging critical roles of Fe-S clusters in DNA replication and repair. Jill O. Fuss, Chi-Lin Tsai, Justin P. Ishida, John A. Tainer, Biochimica et Biophysica Acta (BBA) - Molecular Cell Research, Available online 2 February 2015, ISSN 0167-4889, http://dx.doi.org/10.1016/j.bbamcr.2015.01.018.

Cellular Iron Uptake, Trafficking and Metabolism: Key Molecules and Mechanisms and their Roles in Disease

Cellular Iron Uptake, Trafficking and Metabolism: Key Molecules and Mechanisms and their Roles in Disease. D.J.R. Lane, A.M. Merlot, M.L.-H. Huang, D.-H. Bae, P.J. Jansson, S. Sahni, D.S. Kalinowski, D.R. Richardson, Biochimica et Biophysica Acta (BBA) - Molecular Cell Research, Available online 4 February 2015, ISSN 0167-4889, http://dx.doi.org/10.1016/j.bbamcr.2015.01.021.

Possible roles of frataxin in iron homeostasis under the prespective of the Friedreich's ataxia

Iron Regulatory Protein 1 Sustains Mitochondrial Iron Loading and Function in Frataxin Deficiency

Iron Regulatory Protein 1 Sustains Mitochondrial Iron Loading and Function in Frataxin Deficiency. Alain Martelli, Stéphane Schmucker, Laurence Reutenauer, Jacques R.R. Mathieu, Carole Peyssonnaux, Zoubida Karim, Hervé Puy, Bruno Galy, Matthias W. Hentze, Hélène Puccio, Cell Metabolism, Volume 21, Issue 2, 3 February 2015, Pages 311-322, ISSN 1550-4131, http://dx.doi.org/10.1016/j.cmet.2015.01.010.