Frataxin overexpressing mice
Copyright policy )Frataxin overexpressing mice
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. Iron metabolism parameters in tgFxn were normal and no signs of ataxia or other obvious abnormalities were observed, indicating that overexpression of frataxin in mouse is innocuous. Several hypotheses for frataxin function were evaluated in tgFxn mice. In particular, we observed that TgFxn mice show an altered response during hematopoietic differentiation, suggesting that frataxin may directly affect heme synthesis.
- Université Libre de Bruxelles Belgium
- Department of Medicine Italy
- INSTITUTO DE BIOLOGIA MOLECULAR E CELULAR-IBMC Portugal
- Erasmus Hospital Belgium
- University of Montreal Canada
tgFxn, FA, FA, Friedreich ataxia, Iron-Binding Proteins -- genetics, Iron, Mice, Transgenic, Heme, Hematopoiesis -- genetics, doxorubicin, Polymerase Chain Reaction, Transgenic, PHZ, phenylhydrazine, iron-sulfur cluster, Mice, ISC, Iron-Binding Proteins, Animals, Humans, Transferrin -- metabolism, ISC, iron-sulfur cluster, PHZ, Iron -- metabolism, DNA Primers, DOX, Frataxin, Transferrin, Sciences bio-médicales et agricoles, tgFxn, transgenic mice overexpressing frataxin, DOX, doxorubicin, transgenic mice overexpressing frataxin, Mice, Mutant Strains, Hematopoiesis, Mutant Strains, Phenylhydrazine, Friedreich ataxia, Hematocrit, Friedreich Ataxia -- blood, Doxorubicin, Friedreich Ataxia, Friedreich Ataxia -- genetics, Erythrocyte Count
tgFxn, FA, FA, Friedreich ataxia, Iron-Binding Proteins -- genetics, Iron, Mice, Transgenic, Heme, Hematopoiesis -- genetics, doxorubicin, Polymerase Chain Reaction, Transgenic, PHZ, phenylhydrazine, iron-sulfur cluster, Mice, ISC, Iron-Binding Proteins, Animals, Humans, Transferrin -- metabolism, ISC, iron-sulfur cluster, PHZ, Iron -- metabolism, DNA Primers, DOX, Frataxin, Transferrin, Sciences bio-médicales et agricoles, tgFxn, transgenic mice overexpressing frataxin, DOX, doxorubicin, transgenic mice overexpressing frataxin, Mice, Mutant Strains, Hematopoiesis, Mutant Strains, Phenylhydrazine, Friedreich ataxia, Hematocrit, Friedreich Ataxia -- blood, Doxorubicin, Friedreich Ataxia, Friedreich Ataxia -- genetics, Erythrocyte Count
selected citations These citations are derived from selected sources.This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).24 popularity This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.Average influence This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).Top 10% impulse This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.Top 10%
Citations provided by BIP!Popularity provided by BIP!