Publications by authors named "Chantal Lagresle"
Background: Common variable immunodeficiency (CVID) is characterized by infections and hypogammaglobulinemia. Neutropenia is rare during CVID.
Methods: The French DEFI study enrolled patients with primary hypogammaglobulinemia.
Article Synopsis
- Severe combined immunodeficiencies (SCID) are diseases where the immune system doesn't work well, and gene therapy is being tested to help treat them.
- Gene therapy has worked for most patients, with many showing long-term improvement for up to 6 years.
- However, there have been serious complications in some patients, leading to leukemia in three cases, which shows that while gene therapy is promising, safety must be improved.
Studies of severe combined immunodeficiency (SCID), a group of rare monogenic disorders, have provided key findings about the physiology of immune system development. The common characteristic of these diseases is the occurrence of a block in T cell differentiation, always associated with a direct or indirect impairment of B cell immunity. The resulting combined immunodeficiency is responsible for the clinical severity of SCID, which, without treatment, leads to death within the first year of life.
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- * The study shows a diverse array of T-cell receptors in the patients, suggesting that the genetically corrected cells are not only effective but also varied in their functions.
- * Analysis of cell insertion sites revealed that the treatment targets very early progenitor cells capable of self-renewal, indicating long-term potential for immune system restoration.
Article Synopsis
- The assembly of immunoglobulin (Ig) genes is limited to B cells, while T cell receptors (TCR) rearrange in T cells due to specific regulatory elements.
- Researchers replaced part of the TCR beta-chain locus with a B cell enhancer, which allowed rearrangements in both T and B cells, revealing insights into lineage-specific recombination.
- Despite the change allowing recombination in both cell types, TCR beta allelic exclusion remained effective in the mutated T cells, indicating that regulatory elements play a crucial role in directing recombination patterns.
In the last 30 years, allogeneic bone marrow transplantation has become the treatment of choice for many hematologic malignancies or inherited disorders and a number of changes have been registered in terms of long-term survival rate of transplanted patients as well as of available sources of hematopoietic stem cell (HSC). In parallel to the publication of better results in HSC transplantation, several recent discoveries have opened a scientific and ethical debate on the therapeutical potential of stem cells isolated from adult or embryonic tissues. One of the major discoveries in this field is the capacity of bone marrow-derived stem cells to treat a genetic liver disease in a mouse model, thus justifying the concept of transdifferentiation of adult stem cell and raising hopes on its possible therapeutical applications.
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- T cell development in the thymus involves critical steps of differentiation and selection, with the regulation of thymocyte proliferation being essential for a functional immune system and preventing cancer.
- The gene p16(INK4a) acts as a cell cycle inhibitor, and its deletion is associated with T cell cancers, enhancing thymocyte expansion in studies with mice.
- In transgenic mice expressing human p16(INK4a), its forced expression halted early T cell differentiation without affecting certain T cell types, indicating that p16(INK4a) impedes pre-TCR-mediated growth and survival of developing thymocytes.