期刊
AMERICAN JOURNAL OF HEMATOLOGY
卷 73, 期 2, 页码 115-120出版社
WILEY-LISS
DOI: 10.1002/ajh.10311
关键词
adhesion; integrins; locomotion; neutrophils; motility
类别
资金
- NIAID NIH HHS [AI-43558, AI-20065, AI-10493] Funding Source: Medline
We have defined the defect in a child with severe leukocyte adhesion deficiency-1 (LAD) as resulting from a single amino acid shift in CD18 (from a C to T mutation at position 533) that prevents heterodimerization with the CD11 antigens to produce beta(2) integrins-the first reported patient homozygous for this defect. Although beset by frequent infections, the patient has survived to adolescence despite the lack of these important adhesion molecules. Consistent with his clinical course is the ability of his PMN to respond chemotactically in slide preparations, albeit with difficulty because of their poor purchase on substrate. The operant adhesins are unknown; his polymorphonuclear leukocytes (PMN) remain chemotactically responsive in the presence of antibodies to alphavbeta(3) and beta(1) integrins and to integrin-associated protein (IAP). These findings indicate that not all patients with severe LAD are candidates for early bone marrow transplantation. (C) 2003 Wiley-Liss, Inc.
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