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Institution

Paris Descartes University

GovernmentParis, France
About: Paris Descartes University is a government organization based out in Paris, France. It is known for research contribution in the topics: Population & Transplantation. The organization has 20987 authors who have published 37456 publications receiving 1206222 citations. The organization is also known as: Université Paris V-Descartes & Université de Paris V.


Papers
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Journal ArticleDOI
TL;DR: Observations suggest that ActRIIA ligand traps may have therapeutic relevance in β-thalassemia by suppressing the deleterious effects of GDF11, a cytokine which blocks terminal erythroid maturation through an autocrine amplification loop involving oxidative stress and α-globin precipitation.
Abstract: The pathophysiology of ineffective erythropoiesis in b-thalassemia is poorly understood. We report that RAP-011, an activin receptor IIA (ActRIIA) ligand trap, improved ineffective erythropoiesis, corrected anemia and limited iron overload in a mouse model of b-thalassemia intermedia. Expression of growth differentiation factor 11 (GDF11), an ActRIIA ligand, was increased in splenic erythroblasts from thalassemic mice and in erythroblasts and sera from subjects with b-thalassemia. Inactivation of GDF11 decreased oxidative stress and the amount of a-globin membrane precipitates, resulting in increased terminal erythroid differentiation. Abnormal GDF11 expression was dependent on reactive oxygen species, suggesting the existence of an autocrine amplification loop in b-thalassemia. GDF11 inactivation also corrected the abnormal ratio of immature/mature erythroblasts by inducing apoptosis of immature erythroblasts through the Fas-Fas ligand pathway. Taken together, these observations suggest that ActRIIA ligand traps may have therapeutic relevance in b-thalassemia by suppressing the deleterious effects of GDF11, a cytokine which blocks terminal erythroid maturation through an autocrine amplification loop involving oxidative stress and a-globin precipitation.

261 citations

Journal ArticleDOI
TL;DR: It is concluded that the preservation of NK cell features until very advanced age may contribute to longevity and successful aging.
Abstract: Summary Aging is generally associated with an increased predisposition to infectious diseases and cancers, related in part to the development of immune senescence, a process that affects all cell compartments of the immune system. Although many studies have investigated the effects of age on natural killer (NK) cells, their conclusions remain controversial because the diverse health status of study subjects resulted in discordant findings. To clarify this situation, we conducted the first extensive phenotypic and functional analysis of NK cells from healthy subjects, comparing NK cells derived from newborn (cord blood), middle-aged (18–60 years), old (60–80 years), and very old (80–100 years) subjects. We found that NK cells in cord blood displayed specific features associated with immaturity, including poor expression of KIR and LIR-1/ILT-2 and high expression of both NKG2A and IFN-γ. NK cells from older subjects, on the other hand, preserved their major phenotypic and functional characteristics, but with their mature features accentuated. These include a profound decline of the CD56bright subset, a specific increase in LIR-1/ILT-2, and a perfect recovering of NK-cell function following IL2-activation in very old subjects. We conclude that the preservation of NK cell features until very advanced age may contribute to longevity and successful aging.

261 citations

Journal ArticleDOI
TL;DR: Findings identify a long-sought-after critical component of the nephron stem cell niche and hold promise for long-term culture and utilization of these progenitors in vitro.

261 citations

Journal ArticleDOI
TL;DR: Two new methods for the three-dimensional denoising of magnetic resonance images that exploit the sparseness and self-similarity properties of the images are proposed, making them usable in most clinical and research settings.

261 citations


Authors

Showing all 21023 results

NameH-indexPapersCitations
Guido Kroemer2361404246571
Cyrus Cooper2041869206782
Jean-Laurent Casanova14484276173
Alain Fischer14377081680
Maxime Dougados134105469979
Carlos López-Otín12649483933
Giuseppe Viale12374072799
Thierry Poynard11966864548
Lorenzo Galluzzi11847771436
Shahrokh F. Shariat118163758900
Richard E. Tremblay11668545844
Olivier Hermine111102643779
Yehezkel Ben-Ari11045944293
Loïc Guillevin10880051085
Gérard Socié10792044186
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Performance
Metrics
No. of papers from the Institution in previous years
YearPapers
20238
202279
20211,082
20201,994
20193,298
20183,323