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Showing 1–6 of 6 results
Advanced filters: Author: Jean-Antoine Ribeil Clear advanced filters

  • The heat-shock protein Hsp70 can regulate the development of red blood cells, or erythropoiesis. Hsp70 interacts with GATA-1 and protects it from cleavage, thus controlling the differentiation of erythrocytes by its presence in the nucleus.

    • Jean-Antoine Ribeil
    • Yael Zermati
    • Olivier Hermine
    Research
    Nature
    Volume: 445, P: 102-105

  • The final report of the phase 1/2 lentiviral gene therapy trial in severe β-hemoglobinopathies, HGB-205, shows a long-term safety profile and sustained reduction of transfusion requirements in patients with β-thalassemia and of sickle cell crises in patients with sickle cell disease.

    • Elisa Magrin
    • Michaela Semeraro
    • Marina Cavazzana
    Research
    Nature Medicine
    Volume: 28, P: 81-88

  • In human β-thalassaemiaerythroblasts, HSP70 is sequestered in the cytoplasm by the excess of free α-globin chains and can no longer protect the master transcriptional factor of erythropoiesis GATA-1 from caspase-3 cleavage; transduction of a nuclear-targeted HSP70 or a caspase-3 uncleavable GATA-1 mutant restored maturation of erythropoiesis.

    • Jean-Benoît Arlet
    • Jean-Antoine Ribeil
    • Geneviève Courtois
    Research
    Nature
    Volume: 514, P: 242-246

  • Michael Dussiot et al. show that an activin receptor IIA ligand trap ameliorates anemia in a mouse model of β-thalassemia by blocking the deleterious effects of GDF11. Mechanistically, GDF11 inactivation reversed ineffective erythropoiesis by promoting terminal erythroblast differentiation and by inducing apoptosis of immature erythroblasts. Also in this issue, Rajasekhar Suragani et al. show related findings using a modified activin receptor IIB ligand trap.

    • Michael Dussiot
    • Thiago T Maciel
    • Ivan C Moura
    Research
    Nature Medicine
    Volume: 20, P: 398-407

  • Séverine Coulon et al. identify a new mechanism regulating red blood cell production through transferrin receptor engagement. By binding this receptor on erythroblasts, the polymeric form of immunoglobin A1 (pIgA1) or iron-loaded transferrin acts in conjunction with erythropoietin to promote erythroblast maturation. Administration of either pIgA1 or iron-loaded transferrin accelerated recovery from anemia in mice, suggesting that these findings may have therapeutic implications.

    • Séverine Coulon
    • Michaël Dussiot
    • Ivan C Moura
    Research
    Nature Medicine
    Volume: 17, P: 1456-1465