Abstract
Leukocyte adhesion deficiency (LAD) is an inherited disorder of leukocyte function caused by derangements in CD18 expression. The genetic and functional abnormalities in a lymphocyte cell line from a patient with LAD have been corrected by retrovirus-mediated transduction of a functional CD18 gene. Lymphocytes from patients with LAD were exposed to CD18-expressing retrovirus and enriched for cells that express CD11a and CD18 (LFA-1) on the cell surface. Molecular and functional analyses of these cells revealed (i) one copy of proviral sequence per cell, (ii) viral-directed CD18 RNA that exceeded normal endogenous levels, (iii) normal quantities of CD11a and CD18 protein on the cell surface, and (iv) reconstitution of LFA-1-dependent adhesive function.
Publication types
-
Research Support, Non-U.S. Gov't
-
Research Support, U.S. Gov't, P.H.S.
MeSH terms
-
Animals
-
Antigens, CD
-
Antigens, Differentiation / genetics
-
Antigens, Differentiation / immunology
-
CD18 Antigens
-
Cell Aggregation
-
Cell Line
-
Cell Line, Transformed
-
Gene Expression
-
Genetic Therapy
-
Genetic Vectors
-
Herpesvirus 4, Human
-
Humans
-
Leukocyte-Adhesion Deficiency Syndrome*
-
Lymphocyte Function-Associated Antigen-1
-
Lymphocytes / immunology
-
Membrane Glycoproteins
-
Mice
-
Nucleic Acid Hybridization
-
Receptors, Leukocyte-Adhesion / genetics
-
Receptors, Leukocyte-Adhesion / immunology
-
Retroviridae / genetics*
-
Tetradecanoylphorbol Acetate / pharmacology
-
Transfection*
Substances
-
Antigens, CD
-
Antigens, Differentiation
-
CD18 Antigens
-
Lymphocyte Function-Associated Antigen-1
-
Membrane Glycoproteins
-
Receptors, Leukocyte-Adhesion
-
Tetradecanoylphorbol Acetate