Neutrophil adhesion in leukocyte adhesion deficiency syndrome type 2.

M L Phillips; B R Schwartz; A Etzioni; R Bayer; H D Ochs; J C Paulson; J M Harlan

doi:10.1172/JCI118361

Cytel Corporation, San Diego, California 92121, USA.

Find articles by Phillips, M. in: PubMed | Google Scholar

Cytel Corporation, San Diego, California 92121, USA.

Find articles by Schwartz, B. in: PubMed | Google Scholar

Cytel Corporation, San Diego, California 92121, USA.

Find articles by Etzioni, A. in: PubMed | Google Scholar

Cytel Corporation, San Diego, California 92121, USA.

Find articles by Bayer, R. in: PubMed | Google Scholar

Cytel Corporation, San Diego, California 92121, USA.

Find articles by Ochs, H. in: PubMed | Google Scholar

Cytel Corporation, San Diego, California 92121, USA.

Find articles by Paulson, J. in: PubMed | Google Scholar

Cytel Corporation, San Diego, California 92121, USA.

Find articles by Harlan, J. in: PubMed | Google Scholar

Published in Volume 96, Issue 6 on December 1, 1995
J Clin Invest. 1995;96(6):2898–2906. https://doi.org/10.1172/JCI118361.
© 1995 The American Society for Clinical Investigation

Published December 1, 1995 - Version history

We have previously reported a newly discovered congenital disorder of neutrophil adhesion, leukocyte adhesion deficiency syndrome type 2 (LAD II). The clinical manifestations of this syndrome are similar to those seen in the classic leukocyte adhesion deficiency syndrome, now designated type 1 (LAD I), but the two syndromes differ in the molecular basis of their adhesion defects. LAD I is caused by a deficiency in the CD18 integrin adhesion molecules while LAD II patients are deficient in expression of sialyl-Lewis X (SLeX), a carbohydrate ligand for selectins. In this report we demonstrate that neutrophils from a LAD II patient bind minimally or not at all to recombinant E-selectin, purified platelet P-selectin, or P-selectin expressed on histamine-activated human umbilical vein endothelial cells, but have normal levels of L-selectin and CD11b/CD18 integrin, and adhere to and migrate across endothelium when CD11b/CD18 is activated. We compare LAD I and LAD II patient neutrophil function in vitro, demonstrating that integrin and selectin adhesion molecules have distinct but interdependent roles in neutrophil adhesion during an inflammatory response.

Images.