Leukocyte adhesion deficiency
Golgi nucleotide sugar transport and leukocyte adhesion deficiency II
... new adhesion deficiency syndrome was described in two unrelated Arab boys, then 3 and 5 years old, each the offspring of a consanguineous mating (1, ...described leukocyte adhesion ...
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A novel leukocyte adhesion deficiency caused by expressed but nonfunctional β2 integrins Mac 1 and LFA 1
... the leukocyte adhesion deficiency (LAD)-1 syndrome, there is diminished expression of b2(CD18) ...intercellular adhesion molecule (ICAM)-1, respectively, or to display a b2-integrin activation ...
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Transfection of cells from patients with leukocyte adhesion deficiency with an integrin beta subunit (CD18) restores lymphocyte function associated antigen 1 expression and function
... Leukocyte adhesion deficiency (LAD) is an inherited immunodeficiency disease that is characterized by the deficient expression of the leukocyte adhesion glycoproteins lymphocyte ...
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Mice lacking the signaling molecule CalDAG GEFI represent a model for leukocyte adhesion deficiency type III
... Single gene mutations in b integrins can account for functional defects of individual cells of the hematopoietic system. In humans, mutations in b 2 integrin lead to leukocyte adhesion deficiency ...
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Leukocyte adhesion deficiency type 1 (LAD 1)/variant A novel immunodeficiency syndrome characterized by dysfunctional beta2 integrins
... Leukocyte adhesion deficiency (LAD) is characterized by the inability of leukocytes, in particular neutrophilic granulocytes, to emigrate from the bloodstream towards sites of ...
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Neutrophil adhesion in leukocyte adhesion deficiency syndrome type 2
... classic leukocyte adhesion deficiency syndrome, now designated type 1 (LAD I), but the two syndromes differ in the molecular basis of their adhesion ...a deficiency in the CD18 integrin ...
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Screening for bovine leukocyte adhesion deficiency, deficiency of uridine monophosphate synthase, complex vertebral malformation, bovine citrullinaemia, and factor XI deficiency in Holstein cows reared in Turkey
... bovine leukocyte adhesion deficiency (BLAD), deficiency of uridine monophosphate synthase (DUMPS), complex vertebral malformation (CVM), bovine citrullinaemia (BC), and factor XI ...
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A novel method for rapid and reliable detection of complex vertebral malformation and bovine leukocyte adhesion deficiency in Holstein cattle
... bovine leukocyte adhesion deficiency (BLAD) are two autosomal recessive lethal genetic defects frequently occurring in Holstein cattle, identifiable by single nucleotide ...
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Allogeneic Hematopoietic Stem-Cell Transplantation for Leukocyte Adhesion Deficiency
... OBJECTIVES. Leukocyte adhesion deficiency is a rare primary immune disorder caused by defects of the CD18  -integrin molecule on immune cells. The condition usually presents in early infancy and is ...
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The Biophysics Of Leukocyte Adhesion Deficiency
... The leukocyte adhesion cascade has evolved to slow and stop cells to allow this ...of leukocyte adhesion can be maintained using varying ratios of the two ...cellular adhesion. We ...
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In vivo behavior of neutrophils from two patients with distinct inherited leukocyte adhesion deficiency syndromes
... Flow cytometric analyses of surface expression of CD1 8, L-selectin, and sialyl-Lewisx sLex on neutrophils from a healthy donor control and two patients with leukocyte adhesion deficienc[r] ...
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Leukocyte Adhesion Deficiency Type II is a generalized defect of de novo GDP fucose biosynthesis Endothelial cell fucosylation is not required for neutrophil rolling on human nonlymphoid endothelium
... it has also been proposed that L-selectin can recognize carbo- hydrates on endothelial cells via its lectin domain (45, 46). The carbohydrates involved in this latter interaction have not been identified. However, it is ...
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An in vivo animal model of gene therapy for leukocyte adhesion deficiency
... Table L Cell Surface Expression ofHuman CD18 and Various Murine Epitopes on Nucleated Cells from the Tissues ofMouse 15-6, Six Months after Bone Marrow Transplantation Blood.. Cells were[r] ...
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Detection of recessive mutations (BLAD and CVM) in Holstein-friesian cattle population in Republic of Macedonia
... Bovine Leukocyte Adhesion Deficiency (BLAD) and Complex Vertebral Malformation (CVM) are recessively inherited diseases in Holstein cattle. They are both lethal in homozygous state while the ...
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The effect of edaravone to leukocyte rolling and adhesion molecule expression in microvessels
... accumulating leukocyte led to increasing the number of rolling ...rolling leukocyte in inflammation model was significantly increased com- pared with non-inflammation model, and an increase in this ...
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Sickle cell disease: old discoveries, new concepts, and future promise
... Intravenous gammaglobulins are currently under clinical evaluation following a study demonstrating a dosedependent reduction in leukocyte adhesion and in the number of interactions betwe[r] ...
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Endothelial cell phenotypes in the rheumatoid synovium: activated, angiogenic, apoptotic and leaky
... In RA, endothelial cells in the synovium are generally held to play a central role in the pathophysiology. The cells achieve this in several ways. First, as a component of blood vessels in the subintima, endothelial ...
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Platelet P2Y(12) Inhibitors Reduce Systemic Inflammation and Its Prothrombotic Effects in an Experimental Human Model
... on leukocyte production of cytokines, leukocyte sequestration, platelet-leukocyte aggregate formation and subsequent changes in fibrin clot ultrastructure point to a substantial role for platelets in ...
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Selectins
... Human monocytes bind to two cytokine-induced adhesive ligands on cultured human endothelial cells: endothelial-leukocyte adhesion molecule-I and vascular cell adhesion molecule-l.. Four [r] ...
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Endothelial cells and immune cell migration
... In humans, oral methoxypsoralen combined with intra- articular UV-A irradiation downregulated ICAM-1, VCAM-1 and E-selectin expression in the RA synovium [54]. Gold salts inhibited synovial E-selectin expression in RA ...
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