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Sökning: WFRF:(Materna Thomas)

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1.
  • Lévy, Romain, et al. (författare)
  • Human CARMIL2 deficiency underlies a broader immunological and clinical phenotype than CD28 deficiency.
  • 2023
  • Ingår i: The Journal of experimental medicine. - : Rockefeller University Press. - 1540-9538 .- 0022-1007. ; 220:2
  • Tidskriftsartikel (refereegranskat)abstract
    • Patients with inherited CARMIL2 or CD28 deficiency have defective T cell CD28 signaling, but their immunological and clinical phenotypes remain largely unknown. We show that only one of three CARMIL2 isoforms is produced and functional across leukocyte subsets. Tested mutant CARMIL2 alleles from 89 patients and 52 families impair canonical NF-κB but not AP-1 and NFAT activation in T cells stimulated via CD28. Like CD28-deficient patients, CARMIL2-deficient patients display recalcitrant warts and low blood counts of CD4+ and CD8+ memory T cells and CD4+ TREGs. Unlike CD28-deficient patients, they have low counts of NK cells and memory B cells, and their antibody responses are weak. CARMIL2 deficiency is fully penetrant by the age of 10 yr and is characterized by numerous infections, EBV+ smooth muscle tumors, and mucocutaneous inflammation, including inflammatory bowel disease. Patients with somatic reversions of a mutant allele in CD4+ T cells have milder phenotypes. Our study suggests that CARMIL2 governs immunological pathways beyond CD28.
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2.
  • Litaize, Olivier, et al. (författare)
  • New results in the modeling of fission and radiative neutron capture with FIFRELIN
  • 2023
  • Ingår i: 15TH INTERNATIONAL CONFERENCE ON NUCLEAR DATA FOR SCIENCE AND TECHNOLOGY, ND2022. - : EDP Sciences.
  • Konferensbidrag (refereegranskat)abstract
    • The FIFRELIN Monte Carlo code has been upgraded recently by adding new capabilities, additional models and updated databases. Several examples are presented in this work as well as three different applications related to the prompt component in fission, gamma cascades from thermal neutron capture and the delayed component in fission associated to the time evolution of decay heat.
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