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- W2000066430 abstract "Severe combined immunodeficiency disease (SCID) represents a syndrome of a heterogeneous group of congenital disorders of lymphocyte differentiation. These disorders are associated with profound deficiencies of both humoral and cell-mediated immunity. Several forms of SCID have been characterized by normal levels of adenosine deaminase and intrinsically functional B cells in a number of in vitro assays. This group of patients has been considered within the framework of a proposed model for normal T-cell differentiation. In this model, the stepwise progression in T-cell maturation is characterized by the orderly acquisition of responsiveness to distinct triggering agents, including direct contact with thymic epithelial cells, epithelial-derived products, thymosin and theophylline. Failure to complete these stages can lead to a deficiency of T-cell function and cell-mediated immunity and consequently result in a deficiency of humoral immunity. This approach reveals the potential for new attempts at therapeutic reconstitution of this complex disorder." @default.
- W2000066430 created "2016-06-24" @default.
- W2000066430 creator A5023960478 @default.
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- W2000066430 date "1982-12-01" @default.
- W2000066430 modified "2023-09-23" @default.
- W2000066430 title "Differentiation of precursor T lymphocytes in man and delineation of the selective abnormalities in severe combined immune deficiency disease" @default.
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- W2000066430 doi "https://doi.org/10.1016/0090-1229(82)90195-7" @default.
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