...Hernan G. Gentili; María Florencia Pignataro; Justo Olmos; María Florencia Pavan; Lorena Itatí Ibañez; Javier Santos; Francisco Velazquez Duarte In this paper, we describe the development of a Dictyostelium discoideum strain deficient in frataxin protein (FXN). We investigated the conservation...

...Elena Britti; Fabien Delaspre; A. Sanz-Alcázar; Marta Medina-Carbonero; Marta Llovera; Rosa Purroy; Stefka Mincheva-Tasheva; Jordi Tamarit; Joaquim Ros Friedreich ataxia (FA) is a neurodegenerative disease caused by the deficiency of frataxin, a mitochondrial protein. In primary cultures of dorsal...

...Heeyong Yoon; Simon A. B. Knight; Alok Pandey; Jayashree Pain; Yan Zhang; Debkumar Pain; Andrew Dancis Frataxin is a conserved mitochondrial protein, and deficiency underlies the neurodegenerative disease Friedreich's ataxia. Frataxin interacts with the core machinery for Fe–S cluster assembly...

...Darius J. R. Lane; Michael Li-Hsuan Huang; Samantha Ting; Sutharshani Sivagurunathan; Des R. Richardson FRDA (Friedreich's ataxia) is a debilitating mitochondrial disorder leading to neural and cardiac degeneration, which is caused by a mutation in the frataxin gene that leads to decreased frataxin...

...Dominika Sliwa; Julien Dairou; Jean-Michel Camadro; Renata Santos Friedreich's ataxia is a hereditary neurodegenerative disease caused by reduced expression of mitochondrial frataxin. Frataxin deficiency causes impairment in respiratory capacity, disruption of iron homoeostasis and hypersensitivity...

...Heeyong Yoon; Ramesh Golla; Emmanuel Lesuisse; Jayashree Pain; Jason E. Donald; Elise R. Lyver; Debkumar Pain; Andrew Dancis Frataxin is a conserved mitochondrial protein deficient in patients with Friedreich's ataxia. Frataxin has been implicated in control of iron homoeostasis and Fe–S cluster...

... malignant growth. Recent evidence suggests that specific DNA-repair enzymes contain ISCs (iron–sulfur clusters). The nuclearencoded protein frataxin is essential for the mitochondrial biosynthesis of ISCs. Frataxin deficiency causes a neurodegenerative disorder named Friedreich's ataxia in humans. Various...

...Ana R. Correia; Tao Wang; Elizabeth A. Craig; Cláudio M. Gomes Frataxin is a highly conserved mitochondrial protein whose deficiency in humans results in Friedreich's ataxia (FRDA), an autosomal recessive disorder characterized by progressive ataxia and cardiomyopathy. Although its cellular...

...Darius J. R. Lane; Des R. Richardson What are the structural implications for iron binding by frataxin, the mitochondrial protein whose decreased expression results in Friedreich's ataxia? Though frataxin has been shown to be essential for proper handling of iron within mitochondria (e.g. for iron...

...Ana R. Correia; Salvatore Adinolfi; Annalisa Pastore; Cláudio M. Gomes The neurodegenerative disorder FRDA (Friedreich's ataxia) results from a deficiency in frataxin, a putative iron chaperone, and is due to the presence of a high number of GAA repeats in the coding regions of both alleles...