Results 81 to 90 of about 389 (134)

Cisplatin May Induce Frataxin Expression

Journal of Nippon Medical School, 2003
Cisplatin is a widely used drug in cancer chemotherapy and resistance to cisplatin is a major limitation for its successful application. Intracellular inactivation of cisplatin and detoxification of reactive oxygen species (ROS) by glutathione (a crucial cellular antioxidant) is a mechanism for cisplatin resistance.
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Functional characterization of Frataxin isoforms and mechanisms of regulation of frataxin expression

, 2019
Esta tesis tiene embargado el acceso al texto completo hasta el 04-01 ...
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Reply to “Frataxin fracas” [PDF]

Nature Genetics, 1997
Susan Chamberlain, Mark Pook, Jaime Carvajal, Kitm Doudney, Renate Hillermann   +4 more
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Frataxin (FXN; FRDA) [PDF]

Science-Business eXchange, 2014
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Frataxin overexpressing mice

FEBS Letters, 2004
Friedreich ataxia, the most common autosomal recessive ataxia, is caused by frataxin deficiency. Reduction of frataxin has been associated with iron accumulation and sensitivity to iron induced oxidative stress. To better understand the function of frataxin, transgenic mice (tgFxn) overexpressing human frataxin were generated.
Massimo Pandolfo, CARLOS Miranda, Jorge Sequeiros   +2 more
exaly   +4 more sources

Frataxin knockin mouse [PDF]

FEBS Letters, 2002
Friedreich ataxia is the consequence of frataxin deficiency, most often caused by a GAA repeat expansion in intron 1 of the corresponding gene. Frataxin is a mitochondrial protein involved in iron homeostasis. As an attempt to generate a mouse model of the disease, we introduced a (GAA)230 repeat within the mouse frataxin gene by homologous ...
Massimo Pandolfo, CARLOS Miranda, Jorge Sequeiros   +2 more
exaly   +5 more sources

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Frataxin fracas

Nature Genetics, 1997
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Cossee M, Campuzano V, Koutnikova H, Fischbeck K, Mandel JL, Koenig M, Bidichandani SI, Patel PI, Molte MD, Canizares J, DeFrutos R, Pianese L, Cavalcanti F, MONTICELLI A, Cocozza S, Montermini L, Pandolfo M   +16 more
openaire   +4 more sources

Functional studies of frataxin

Acta Paediatrica, 2004
Mitochondria generate adenosine triphosphate (ATP) but also dangerous reactive oxygen species (ROS). One‐electron reduction of dioxygen in the early stages of the electron transport chain yields a superoxide radical that is detoxified by mitochondrial superoxide dismutase to give hydrogen peroxide.
G, Isaya, H A, O'Neill, O, Gakh, S, Park, R, Mantcheva, S M, Mooney   +5 more
openaire   +2 more sources

The kinetics of folding of frataxin

Physical Chemistry Chemical Physics, 2014
The role of the denatured state in protein folding represents a key issue for the proper evaluation of folding kinetics and mechanisms. The yeast ortholog of the human frataxin, a mitochondrial protein essential for iron homeostasis and responsible for Friedreich's ataxia, has been shown to undergo cold denaturation above 0 °C, in the absence of ...
BONETTI, DANIELA, TOTO, ANGELO, Giri R, Morrone A, Sanfelice D, Pastore A, Temussi P, GIANNI, STEFANO, BRUNORI, Maurizio   +8 more
openaire   +5 more sources

Ferrochelatase activity of plant frataxin [PDF]

Biochimie, 2019
Frataxin plays a key role in cellular iron homeostasis of different organisms. It is engaged in several activities at the FeS cluster assembly machinery and it is also involved in heme biosynthesis. In plants, two genes encoding ferrochelatases (FC1 and FC2) catalyze the incorporation of iron into protoporphyrin IX in the last stage of heme synthesis ...
Manuel Balparda, Maria Ayelén Pagani, Diego Gómez-Casati   +2 more
exaly   +4 more sources