The T-cellreceptor complex consists of the and or and variant chains, paired as mutually exclusive heterodimersin association with the invariant chains CD3, , , and . T cellswith and chains are referred to as / T cells, and those with and chains are called / T cells. During development, the CD3protein complex plays an important part in the transition ofthymocytes from CD4CD8 double-negative immatureprecursors to a CD4+CD8+ double-positive stage and finally tothe mature CD4+CD8 or CD4CD8+ single-positiveT cell.1,2,3,4,5 Selective deficiency of CD3 component , , ,or in mice, achieved by gene . . . [Full Text of this Article]
Case Report
Methods
Assays of Phenotype and Function of Peripheral-Blood Lymphocytes
Analysis of Thymic Tissue
Western Blotting
Preparation of RNA, Genomic DNA, and Complementary DNA
Microarray Analysis
Results
Microarray Analysis of Gene Expression in the Thymus of Patient 1
Identification of a Premature Stop Codon in CD3
Proteins of the CD3 Complex in CD3/ Thymocytes
Arrest of CD3/ Thymocytes at the CD4CD8 Stage of Development
The / Lineage in CD3 Deficiency
Discussion
Source Information
From the Divisions of Immunology and Allergy and the Infection, Immunity, Injury and Repair Program, the Research Institute and the Hospital for Sick Children, University of Toronto, Toronto.
Address reprint requests to Dr. Roifman at the Division of Immunology and Allergy and the Infection, Immunity, Injury and Repair Program, Hospital for Sick Children, 555 University Ave., Toronto, ON M5G 1X8, Canada, or at chaim.roifman@sickkids.ca.
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