The Marfan syndrome, a rare disease causing a marked decrease in life expectancy by involving the skeletal, ocular, and cardiovascular systems, is known as a connective tissue disorder that is inherited autosomal dominant. The cardiovascular complications directly related to the cause of death are associated with more than 90% of the Marfan syndrome. Aortic aneurysm with rupture could occur because of dilatation of aorta due to defect of media. Aortic and mitral insufficiency, mitral valve prolapse, bacterial endocarditis, arrhythmia, and aneurysm of interatrial septum are also frequently observed. A few literatures were reported domestically pertaining to the Marfan syndrome thus far, and there are increased discoveries of cardiovascular complications of the syndrome with the application of echocardiogram. However, the Marfan syndrome with LA myxoma has never been reported both domestically and internationally(INDEX MEDICUS, 1966-1989). Therefore we hereby report a case of the Marfan syndrome with LA myxoma from the observation of a patient who was admitted to Eulji General Hospital at Taejeon i August of 1989.
Aneurysm ; Aorta ; Aortic Aneurysm ; Arrhythmias, Cardiac ; Cardiovascular System ; Cause of Death ; Connective Tissue ; Daejeon ; Dilatation ; Echocardiography* ; Endocarditis, Bacterial ; Hospitals, General ; Humans ; Life Expectancy ; Marfan Syndrome* ; Mitral Valve Insufficiency ; Mitral Valve Prolapse ; Myxoma* ; Rare Diseases ; Rupture

Glucocorticoid receptor (GR) functions as a suppressor of inflammation by inhibiting the expression of many cytokine gene activated by NF-κB. The goal of this study is to investigate the mechanism by which GR repress NF-κB activation in lung epithelial cells. We used A549 and BEAS-2B lung epithelial cell lines. Using IgGκ-NF-κB luciferase reporter gene construct, we found that dexamethasone significantly suppressed TNF-α-induced NF-κB activation and the overexpression of GR showed dose-dependent reduction of TNF-α-induced NF-κB activity in both cell lines. However, DNA binding of NF-κB induced by TNF-α in electromobility shift assay was not inhibited by dexamethasone. Super shift assay with anti-p65 antibody demonstrated the existence of p65 in NF-κB complex induced by TNF-α Western blot showed that IκBα degradation induced by TNF-α was not affected by dexamethasone and IκBκ was not induced by dexamethasone, neither. To evaluate p65 specific transactivation, we adopted co-transfection study of Ga14-p65TA1 or TA2 fusion protein expression system together with 5xGa14-luciferase vector. Co-transfection of GR with Ga14-p65TA1 or TA2 repressed luciferase activity profoundly to the level of 10-20% of p65TA1- or TA2-induced transcriptional activity. And this transrepressional effect was abolished by co-transfection of CBP or SRC-1 expression vectors. These results suggest that Gr-mediated transrepression of NF-κB in lung epithelial cells is through competing for binding to limiting amount of transcriptional coactivators, CBP or SRC-1.
Blotting, Western ; Cell Line ; Dexamethasone* ; DNA ; Epithelial Cells* ; Genes, Reporter ; Inflammation ; Luciferases ; Lung ; NF-kappa B* ; Receptors, Glucocorticoid ; Transcriptional Activation*

Increased expression of a number of proinflammatory genes, including IL-8, is associated with inflammatory conditions such as asthma. Glucocorticoid receptor (GR)beta, one of the GR isoforms, has been suggested to be upregulated in asthma associated with glucocorticoid insensitivity and to work as a dominant negative inhibitor of wild type GRalpha. However, recent data suggest that GRbeta is not a dominant negative inhibitor of GRalpha in the transrepressive process and has its own functional role. We investigated the functional role of GRbeta expression in the suppressive effect of glucocorticoids on tumor necrosis factor (TNF)-alpha-induced IL-8 release in an airway epithelial cell line. GRbeta expression was induced by treatment of epithelial cells with either dexamethasone or TNF-alpha. GRbeta was able to inhibit glucocorticoid-induced transcriptional activation mediated by binding to glucocorticoid response elements (GREs). The suppressive effect of dexamethasone on TNF-alpha-induced IL-8 transcription was not affected by GRbeta overexpression, rather GRbeta had its own weak suppressive activity on TNF-alpha-induced IL-8 expression. Overall histone deacetylase activity and histone acetyltransferase activity were not changed by GRbeta overexpression, but TNF-alpha-induced histone H4 acetylation at the IL-8 promoter was decreased with GRbeta overexpression. This study suggests that GRbeta overexpression does not affect glucocorticoid-induced suppression of IL-8 expression in airway epithelial cells and GRbeta induces its own histone deacetylase activity around IL-8 promoter site.
Acetylation ; Cell Line, Tumor ; Dexamethasone/pharmacology ; Epithelial Cells/metabolism ; *Gene Expression Regulation ; Histones/*metabolism ; Humans ; Interleukin-8/*genetics/metabolism ; Receptors, Glucocorticoid/genetics/*metabolism ; Transcriptional Activation ; Transfection ; Tumor Necrosis Factor-alpha/*antagonists & inhibitors/pharmacology

Increased expression of a number of proinflammatory genes, including IL-8, is associated with inflammatory conditions such as asthma. Glucocorticoid receptor (GR)beta, one of the GR isoforms, has been suggested to be upregulated in asthma associated with glucocorticoid insensitivity and to work as a dominant negative inhibitor of wild type GRalpha. However, recent data suggest that GRbeta is not a dominant negative inhibitor of GRalpha in the transrepressive process and has its own functional role. We investigated the functional role of GRbeta expression in the suppressive effect of glucocorticoids on tumor necrosis factor (TNF)-alpha-induced IL-8 release in an airway epithelial cell line. GRbeta expression was induced by treatment of epithelial cells with either dexamethasone or TNF-alpha. GRbeta was able to inhibit glucocorticoid-induced transcriptional activation mediated by binding to glucocorticoid response elements (GREs). The suppressive effect of dexamethasone on TNF-alpha-induced IL-8 transcription was not affected by GRbeta overexpression, rather GRbeta had its own weak suppressive activity on TNF-alpha-induced IL-8 expression. Overall histone deacetylase activity and histone acetyltransferase activity were not changed by GRbeta overexpression, but TNF-alpha-induced histone H4 acetylation at the IL-8 promoter was decreased with GRbeta overexpression. This study suggests that GRbeta overexpression does not affect glucocorticoid-induced suppression of IL-8 expression in airway epithelial cells and GRbeta induces its own histone deacetylase activity around IL-8 promoter site.
Acetylation ; Cell Line, Tumor ; Dexamethasone/pharmacology ; Epithelial Cells/metabolism ; *Gene Expression Regulation ; Histones/*metabolism ; Humans ; Interleukin-8/*genetics/metabolism ; Receptors, Glucocorticoid/genetics/*metabolism ; Transcriptional Activation ; Transfection ; Tumor Necrosis Factor-alpha/*antagonists & inhibitors/pharmacology

Ulmus davidiana var. japonica Rehder (Urticales: Ulmaceae) (UD) is a tree widespread in northeast Asia. It is traditionally used for anticancer and anti-inflammatory therapy. The present study investigated the effect of an ethanol extract of UD on vascular tension and its underlying mechanism in rats. The dried root bark of UD was ground and extracted with 80% ethanol. The prepared UD extract was used in further analysis. The effect of UD on the cell viability, vasoreactivity and hemodynamics were investigated using propidium iodide staining in cultured cells, isometric tension recording and blood pressure analysis, respectively. Low dose of UD (10~100microg/ml) did not affect endothelial cell viability, but high dose of UD reduced cell viability. UD induced vasorelaxation in the range of 0.1~10microg/ml with an ED50 value of 2microg/ml. UD-induced vasorelaxation was completely abolished by removal of the endothelium or by pre-treatment with L-NAME, an inhibitor of nitric oxide synthase. UD inhibited calcium influx induced by phenylephrine and high K+ and also completely abolished the effect of L-NAME. Intravenous injection of UD extracts (10~100 mg/kg) decreased arterial and ventricular pressure in a dose-dependent manner. Moreover, UD extracts reduced the ventricular contractility (+dP/dt) in anesthetized rats. However, UD-induced hypotensive actions were minimized in L-NAME-treated rats. Taken together, out results showed that UD induced vasorelaxation and has antihypertensive properties, which may be due the activation of nitric oxide synthase in endothelium.
Animals ; Asia ; Blood Pressure ; Calcium ; Cell Survival ; Cells, Cultured ; Endothelial Cells ; Endothelium ; Ethanol ; Hemodynamics ; Injections, Intravenous ; NG-Nitroarginine Methyl Ester ; Nitric Oxide Synthase ; Nitric Oxide Synthase Type III ; Phenylephrine ; Propidium ; Rats ; Trees ; Ulmus ; Vasodilation ; Ventricular Pressure