{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,14]],"date-time":"2026-09-14T13:58:14Z","timestamp":1789394294267,"version":"build-2803163510"},"reference-count":90,"publisher":"American Society of Hematology","issue":"3","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2001,2,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Leukocyte adhesion deficiency type I (LAD-1) is a disorder associated with severe and recurrent bacterial infections, impaired extravascular targeting and accumulation of myeloid leukocytes, altered wound healing, and significant morbidity that is caused by absent or greatly diminished surface expression of integrins of the \u03b22 class. We report clinical features and analysis of functions of cells from a patient with a myelodysplastic syndrome and infectious complications similar to those in the severe form of LAD-1, but whose circulating neutrophils displayed normal levels of \u03b22 integrins. Analysis of adhesion of these cells to immobilized ligands and to endothelial cells and assays of cell-cell aggregation and chemotaxis demonstrated a profound defect in adhesion mediated by \u03b22 integrins indicative of a variant form of LAD-1. A novel cell line established from Epstein-Barr virus\u2013transformed lymphoblasts from the subject demonstrated deficient \u03b22 integrin\u2013dependent adhesive function similar to that of the primary leukocytes. In addition, these cells had markedly impaired \u03b21 integrin\u2013dependent adhesion. Sequence analysis and electrophoretic mobility of \u03b21 and \u03b22 proteins from the cell line demonstrated that the defects were not a result of structural abnormalities in the integrin subunit chains themselves and suggest that the adhesive phenotype of these cells is due to one or more abnormalities of inside-out signaling mechanisms that regulate the activity of integrins of these classes. These features define a unique LAD-1 variant syndrome that may reveal important insights that are generally relevant to inside-out signaling of integrins, a molecular process that is as yet incompletely understood.<\/jats:p>","DOI":"10.1182\/blood.v97.3.767","type":"journal-article","created":{"date-parts":[[2002,7,27]],"date-time":"2002-07-27T12:46:47Z","timestamp":1027774007000},"page":"767-776","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":55,"title":["A novel syndrome of variant leukocyte adhesion deficiency involving defects in adhesion mediated by \u03b21 and \u03b22 integrins"],"prefix":"10.1182","volume":"97","author":[{"given":"Estelle S.","family":"Harris","sequence":"first","affiliation":[{"name":"From the Departments of Pediatrics, Internal Medicine, and Experimental Pathology, the Huntsman Cancer Institute, and the Program in Human Molecular Biology and Genetics, the University of Utah School of Medicine, Salt Lake City, UT."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ann O.","family":"Shigeoka","sequence":"additional","affiliation":[{"name":"From the Departments of Pediatrics, Internal Medicine, and Experimental Pathology, the Huntsman Cancer Institute, and the Program in Human Molecular Biology and Genetics, the University of Utah School of Medicine, Salt Lake City, UT."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wenhua","family":"Li","sequence":"additional","affiliation":[{"name":"From the Departments of Pediatrics, Internal Medicine, and Experimental Pathology, the Huntsman Cancer Institute, and the Program in Human Molecular Biology and Genetics, the University of Utah School of Medicine, Salt Lake City, UT."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberta H.","family":"Adams","sequence":"additional","affiliation":[{"name":"From the Departments of Pediatrics, Internal Medicine, and Experimental Pathology, the Huntsman Cancer Institute, and the Program in Human Molecular Biology and Genetics, the University of Utah School of Medicine, Salt Lake City, UT."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen M.","family":"Prescott","sequence":"additional","affiliation":[{"name":"From the Departments of Pediatrics, Internal Medicine, and Experimental Pathology, the Huntsman Cancer Institute, and the Program in Human Molecular Biology and Genetics, the University of Utah School of Medicine, Salt Lake City, UT."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas M.","family":"McIntyre","sequence":"additional","affiliation":[{"name":"From the Departments of Pediatrics, Internal Medicine, and Experimental Pathology, the Huntsman Cancer Institute, and the Program in Human Molecular Biology and Genetics, the University of Utah School of Medicine, Salt Lake City, UT."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guy 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