EXPRESSION, BIOCHEMICAL PROPERTIES, AND BIOLOGY OF FRATAXIN, THE FRIEDREICH’S ATAXIA GENE PRODUCT
- 3 Anni 1997/2000
- 139.443€ Totale Fondi
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Pubblicazioni Scientifiche
- ANTIOXIDANTS & REDOX SIGNALING
Sensitivity of FRDA Lymphoblasts to Salts of Transition Metal Ions
- HUMAN MOLECULAR GENETICS
Frataxin expression rescues mitochondrial dysfunctions in FRDA cells
- HUMAN MOLECULAR GENETICS
Decreased expression of genes involved in sulfur amino acid metabolism in frataxin-deficient cells
- HUMAN MOLECULAR GENETICS
The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis
- JOURNAL OF BIOLOGICAL CHEMISTRY
Yeast and human frataxin are processed to mature form in two sequential steps by the mitochondrial processing peptidase
- JOURNAL OF BIOLOGICAL CHEMISTRY
Two-step processing of human frataxin by mitochondrial processing peptidase - Precursor and intermediate forms are cleaved at different rates
