CXCR4, a therapeutic target in rare immunodeficiencies?

被引：4

作者：

Bignon, Alexandre ^{[1
,2
]}

Biajoux, Vincent ^{[1
,2
]}

Bouchet-Delbos, Laurence ^{[1
,2
]}

Emilie, Dominique ^{[1
,2
,3
]}

Lortholary, Olivier ^{[4
]}

Balabanian, Karl ^{[1
,2
]}

机构：

[1] Univ Paris 11, Lab Cytokines Chimiokines & Immunopathol, UMR S996, F-92140 Clamart, France

[2] INSERM, F-92140 Clamart, France

[3] AP HP, F-92140 Clamart, France

[4] Univ Paris 05, Hop Necker Enfants Malad, Serv Malad Infect & Trop, F-75743 Paris, France

来源：

M S-MEDECINE SCIENCES | 2011年 / 27卷 / 04期

关键词：

IDIOPATHIC CD4+ LYMPHOCYTOPENIA; WHIM SYNDROME PATIENTS; PROGENITOR CELLS; T-LYMPHOCYTES; RECEPTOR; DESENSITIZATION; INFECTION; DISEASE; UPDATE; CXCL12;

D O I：

10.1051/medsci/2011274015

中图分类号：

R-3 [医学研究方法]; R3 [基础医学];

学科分类号：

1001 ;

摘要：

CXCR4, a therapeutic target in rare immunodeficiencies? Currently, more than 200 primary immunodeficiency diseases have been discovered. In most cases, genetic defects affect the expression or the function of proteins involved in immune development and homeostasis. Some orphan immuno-hematological disorders are characterized by an abnormal leukocyte trafficking, a notion predictive of an anomaly of the chemokine/chemokine receptor system. In this review, we focus on recent advances in the characterization of dysfunctions of the CXCL12 (SDF-1)/CXCR4 signaling axis in two rare human immunodeficiencies, one associated with a loss of CXCR4 function, the Idiopathic CDC(+) 1-cell Lymphocytopenia, and the other with a gain of CXCR4 function, the WHIM syndrome.

引用

页码：391 / 397

页数：7

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