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A correction has been published: N Engl J Med 2004;350(17):1803.

Original Article
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Volume 349:1821-1828 November 6, 2003 Number 19
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Effect of CD3{delta} Deficiency on Maturation of {alpha}/ß and {gamma}/{delta} T-Cell Lineages in Severe Combined Immunodeficiency
Harjit K. Dadi, Ph.D., Amos J. Simon, Ph.D., and Chaim M. Roifman, M.D.

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 by Fischer, A.

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The T-cell–receptor complex consists of the {alpha} and {beta} or {gamma} and {delta} variant chains, paired as mutually exclusive heterodimers in association with the invariant chains CD3{gamma}, {delta}, {epsilon}, and {zeta}. T cells with {alpha} and {beta} chains are referred to as {alpha}/{beta} T cells, and those with {gamma} and {delta} chains are called {gamma}/{delta} T cells. During development, the CD3 protein complex plays an important part in the transition of thymocytes from CD4–CD8– double-negative immature precursors to a CD4+CD8+ double-positive stage and finally to the mature CD4+CD8– or CD4–CD8+ single-positive T cell.1,2,3,4,5 Selective deficiency of CD3 component {gamma}, {delta}, {epsilon}, or {zeta} 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{delta}

Proteins of the CD3 Complex in CD3{delta}–/– Thymocytes

Arrest of CD3{delta}–/– Thymocytes at the CD4–CD8– Stage of Development

The {gamma}/{delta} Lineage in CD3{delta} 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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