Publications by authors named "M Sarzotti"

The purpose of this study was to characterize a large group of infants with complete DiGeorge anomaly and to evaluate the ability of thymus transplantation to reconstitute immune function in these infants. DiGeorge anomaly is characterized by varying defects of the heart, thymus, and parathyroid glands. Complete DiGeorge anomaly refers to the subgroup that is athymic (< 1%).

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Article Synopsis
  • CD3zeta is crucial for T-cell receptor assembly and signaling; a patient with severe combined immunodeficiency (SCID) had a mutation in the CD3zeta gene that disrupted this function.
  • The patient's T cells were almost entirely nonfunctional, showing very low levels of CD3epsilon and unique NK cell populations with reduced activity.
  • Experiments showed that the mutated CD3zeta protein could not properly assemble TCR complexes or maintain stability, highlighting how a lack of CD3zeta can lead to SCID in humans.
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Immunizations early in life, when the host is most susceptible to infection, allow protective immunological memory to develop. Decreasing the dose of Cas-Br-E murine leukemia virus when priming neonatal mice results in adult-like, Type 1 protective responses, but the resulting memory cell populations are smaller than after adult priming. After secondary challenge, virus-specific CD8+ memory cell populations expand twice as much in neonate-primed mice as in adult-primed mice.

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Summary: SpA is a web-accessible system for the management, visualization and statistical analysis of T-cell receptor spectratype data. Users upload data from their spectratype analyzers to SpA, which saves the raw data and user-defined supplementary covariates to a secure database. The statistical engine performs several data analyses and statistical summaries.

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