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Animal Model Of Human Disease: Bovine Leukocyte Adhesion Deficiency: Beta2 Integrin Deficiency In Young Holstein Cattle, Marcus E. Kehrli Jr., Mark R. Ackermann, Dale E. Shuster, Martin J. Van Der Maaten, Frank C. Schmalstieg, Donald C. Anderson, Bonnie J. Hughes

Mark R. Ackermann

Leukocyte adhesion deficiency (LAD), is a rare, autosomal recessive disorder caused by a lack or partial absence of a family of leukocyte integrins, Mac-1, LFA-1, and p150,95.1 The leukocyte (32) integrins are glycoproteins essential for normal leukocyte-endothelial cell adherence and emigration. Affected children develop recurrent bacterial infections, persistent leukocytosis, severe hypoplasia of lymphoid tissues and isolated neutrophils, eosinophils, macrophages, and lymphocytes have abnormal function when tested in vitro. Most patients without bone marrow transplants die at an early age.