Leukocyte adhesion deficiency: an inherited defect in the Mac-1, LFA-1, and p150, 95 glycoproteins

DC Anderson, TA Springer - Annual review of medicine, 1987 - annualreviews.org
DC Anderson, TA Springer
Annual review of medicine, 1987annualreviews.org
Leukocyte adhesion deficiency (LAD) is a recently recognized autosomal recessive trait
characterized by recurrent bacterial infections, impaired pus formation and wound healing,
and abnormalities in a wide spectrum of adherence-dependent functions of granulocytes,
monocytes, and lymphoid cells. Features of this disease are attributable to deficiency (or
absence) of cell surface expression of a family of functionally and structurally related
glycoproteins. These include Mac-l (complement receptor type 3), lym phocyte function …
Abstract
Leukocyte adhesion deficiency (LAD) is a recently recognized autosomal recessive trait characterized by recurrent bacterial infections, impaired pus formation and wound healing, and abnormalities in a wide spectrum of adherence-dependent functions of granulocytes, monocytes, and lymphoid cells. Features of this disease are attributable to deficiency (or absence) of cell surface expression of a family of functionally and structurally related glycoproteins. These include Mac-l (complement receptor type 3), lym phocyte function-associated antigen-l (LF AI), and p 150, 95. Defective biosynthesis of the p chain shared by each molecule (comprised of iY. IPI complexes) represents the fundamental molecular basis of this disease. Recognition of the molecular pathogenesis of this disorder has allowed rich insights into the role of cellular adherence reactions in inflammation and host defense.
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