cd8 deficiency is a rare primary immunodeficiency with low or absent peripheral cd8 cells which results from tap deficiency, zap 70 deficiency and cd8 α gene mutation. we report a 14 year old female who presented with a history of recurrent pneumonia, bronchiectasis, otitis, severe varicella, herpetic lesions of mouth, bilateral uveitis, and cataract formation since the age of 8 years. she had ...

As a result of interaction with epithelial cells in the thymic cortex, immature CD4 1 8 1 (double positive, DP) thymocytes express relatively few T cell receptors (TCRs) and contain diminished numbers of coreceptor-associated p56 lck (lck) PTK molecules. As a result, TCR signal transduction in DP thymocytes is significantly impaired, despite its importance for repertoire selection. We report he...

The protein tyrosine kinase, ZAP-70, is pivotally involved in transduction of Ag-binding signals from the TCR required for T cell activation and development. Defects in ZAP-70 result in SCID in humans and mice. We describe an infant with SCID due to a novel ZAP-70 mutation, comparable with that which arose spontaneously in an inbred mouse colony. The patient inherited a homozygous missense muta...

As a result of interaction with epithelial cells in the thymic cortex, immature CD4(+)8(+) (double positive, DP) thymocytes express relatively few T cell receptors (TCRs) and contain diminished numbers of coreceptor-associated p56(lck) (lck) PTK molecules. As a result, TCR signal transduction in DP thymocytes is significantly impaired, despite its importance for repertoire selection. We report ...

Transendothelial migration of activated lymphocytes from the blood into the tissues is an essential step for immune functions. The housekeeping chemokine CXCL12 (or stroma cell-derived factor-1alpha), a highly efficient chemoattractant for T lymphocytes, drives lymphocytes to sites where they are highly likely to encounter antigens. This suggests that cross-talk between the T-cell receptor (TCR...

Recently, a severe combined immunodeficiency syndrome with a deficiency of CD8 + peripheral T cells and a T C R signal transduction defect in peripheral CD4 + T cells was associated with mutations in ZAP-70. Since T C R signaling is required in developmental decisions resulting in mature CD4 (and CD8) T cells, the presence of peripheral CD4 + T cells expressing TCRs incapable of signaling in th...

Recently, a severe combined immunodeficiency syndrome with a deficiency of CD8+ peripheral T cells and a TCR signal transduction defect in peripheral CD4+ T cells was associated with mutations in ZAP-70. Since TCR signaling is required in developmental decisions resulting in mature CD4 (and CD8) T cells, the presence of peripheral CD4+ T cells expressing TCRs incapable of signaling in these pat...

Recently, a severe combined immunodeficiency syndrome with a deficiency of CD8 + peripheral T cells and a T C R signal transduction defect in peripheral CD4 + T cells was associated with mutations in ZAP-70. Since T C R signaling is required in developmental decisions resulting in mature CD4 (and CD8) T cells, the presence of peripheral CD4 + T cells expressing TCRs incapable of signaling in th...