RESEARCH ARTICLE
Upregulation of Mitochondrial Content in Cytochrome c Oxidase Deficient Fibroblasts Aviram Kogot-Levin1, Ann Saada2, Gil Leibowitz1, Devorah Soiferman2, Liza Douiev2, Itamar Raz1, Sarah Weksler-Zangen1* 1 The Diabetes Unit, Department of Internal Medicine, Hadassah-Hebrew University Medical Center, Jerusalem, Israel, 2 Monique and Jacques Roboh Department of Genetic Research and Department of Genetic and Metabolic Diseases, Hadassah-Hebrew University Medical Center, Jerusalem, Israel * [email protected]
a11111
Abstract
OPEN ACCESS Citation: Kogot-Levin A, Saada A, Leibowitz G, Soiferman D, Douiev L, Raz I, et al. (2016) Upregulation of Mitochondrial Content in Cytochrome c Oxidase Deficient Fibroblasts. PLoS ONE 11(10): e0165417. doi:10.1371/journal. pone.0165417 Editor: Guillermo Lo´pez Lluch, Universidad Pablo de Olavide, SPAIN Received: June 28, 2016 Accepted: October 11, 2016 Published: October 25, 2016 Copyright: © 2016 Kogot-Levin et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Cytochrome-c-oxidase (COX) deficiency is a frequent cause of mitochondrial disease and is associated with a wide spectrum of clinical phenotypes. We studied mitochondrial function and biogenesis in fibroblasts derived from the Cohen (CDs) rat, an animal model of COX deficiency. COX activity in CDs-fibroblasts was 50% reduced compared to control rat fibroblasts (P
Upregulation of Mitochondrial Content in Cytochrome c Oxidase Deficient Fibroblasts Aviram Kogot-Levin1, Ann Saada2, Gil Leibowitz1, Devorah Soiferman2, Liza Douiev2, Itamar Raz1, Sarah Weksler-Zangen1* 1 The Diabetes Unit, Department of Internal Medicine, Hadassah-Hebrew University Medical Center, Jerusalem, Israel, 2 Monique and Jacques Roboh Department of Genetic Research and Department of Genetic and Metabolic Diseases, Hadassah-Hebrew University Medical Center, Jerusalem, Israel * [email protected]
a11111
Abstract
OPEN ACCESS Citation: Kogot-Levin A, Saada A, Leibowitz G, Soiferman D, Douiev L, Raz I, et al. (2016) Upregulation of Mitochondrial Content in Cytochrome c Oxidase Deficient Fibroblasts. PLoS ONE 11(10): e0165417. doi:10.1371/journal. pone.0165417 Editor: Guillermo Lo´pez Lluch, Universidad Pablo de Olavide, SPAIN Received: June 28, 2016 Accepted: October 11, 2016 Published: October 25, 2016 Copyright: © 2016 Kogot-Levin et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Cytochrome-c-oxidase (COX) deficiency is a frequent cause of mitochondrial disease and is associated with a wide spectrum of clinical phenotypes. We studied mitochondrial function and biogenesis in fibroblasts derived from the Cohen (CDs) rat, an animal model of COX deficiency. COX activity in CDs-fibroblasts was 50% reduced compared to control rat fibroblasts (P
