Metallothioneins (MT), small molecular weight metal binding proteins are known to play an important protective role against heavy metal toxicity, either as antioxidants or pre-oxidants. However, the mode of metabolic fate of MTs in various metal complexes is not clearly understood. This study was carried out to better understand the mode of selective turnover rate of various form of MT in complexes with different metals. The degradation of in vitro translated mouse 35S-cysteine-MT was examined in lysosomal or cytosolic fractions from mouse liver by gel electrophoresis and autoradiography. Overnight incubations of MT showed extensive proteolysis in the lysosomal fraction but not in cytosolic fractions. However, Cu2+-MT was found to be stable under the same experimental condition. In contrast, Zn did not interfere with MT degradation. These results suggest that lysosomes are chiefly responsible for MT removal and appears to be selective on the metals involved in the MT complex. In vitro, translated, radiolabeled MT provides a suitable substrate for investigating the characteristics of MT degradation.
Animal ; Copper/*metabolism/pharmacology ; Ions ; Liver/drug effects/*metabolism ; Lysosomes/metabolism ; Metallothionein/drug effects/*metabolism ; Mice ; Sulfur Radioisotopes ; Support, Non-U.S. Gov't ; Zinc/*metabolism/pharmacology

No abstract available.
Leukemia, Myelomonocytic, Chronic*

To evaluate the clinical significance of anomalous pancreaticobiliary ductal union(PBDU), we analysed 11 cases(0.19%) of anomalous PBDU among 5675 cases performed ERCP from Jan. 1973 to Aug. 1992. According to the classification of Kimura et al., 4 cases were type 1(P-C union) and 7 cases type 2(C-P union). The length of common channel ranged from 1.5 cm to 3.1 cm(mean 1.9 cm). The common associated disease were choledochal cyst(7 cases), carcinoma of the gallbladder(5 cases) and biliary stone(3 cases). Four cases of carcinoma of the gallbladder were type 1. The incidence of anomalous PBDU was significantly higher in cases with carcinoma of the gallbladder(5 cases among 49 cases) than in case without carcinoma of the gallbladder(6 cases among 5626 cases). And the incidence of gallbladder carcinoma(45%) among the 11 cases of anomalous PBDU was significantly higher than that(0.78%) among those who did not have anomalous PBDU. In conclusion, the common associated diseases with the anomalous PBDU were choledochal cyst, carcinoma of the gallbladder and stone, and the anomalous PBDU may be risk factor in the development of carcinoma of the gllbladder.
Cholangiopancreatography, Endoscopic Retrograde* ; Choledochal Cyst ; Classification ; Gallbladder ; Incidence ; Risk Factors

The coronary arteries are also subject to congenital anomalies of both minor and major consequence. Hemodynamically significant primary anomalies of the coronary arteries are those which alter myocardial perfusion. There are four major types : coronary artery fistula, origin of the left voronary artery from the pulmonary artery, congenital coronary stenosis or atresia and origin of the left or right coronary artery from the opposite sinus of Valsalva with subsequent passage of the vessel between the aorta and right ventricular infundibulum. Minor anomlies are the variation of the origin of the coronary arteries from the aorta with normal distal circulation. We can see more coronary anomalies associated with congenital cardiac anomalies, probably representing a circulatory response to the primary intracardiac defect. We present a total of 76 cases of congenital coronary anomalies, reviewing 3946 cases of angiocardiography or selective coronary arteriography, performed during last 8 years at Sejong General Hospital.
Angiocardiography ; Angiography ; Aorta ; Arteries ; Coronary Stenosis ; Coronary Vessels* ; Fistula ; Hospitals, General ; Perfusion ; Pulmonary Artery ; Sinus of Valsalva

It was difficult to determine the clinical situations of Morgagni diaphragmatic hernias of adults on account of its rarity. The aim of this study was to propose diagnostic and therapeutic guidelines for adult Morgagni diaphragmatic hernias. A computerized `Korean study information co.' and `medical research information center' literature search of domestic articles published since 1950 was carried out. These 10 cases in addition to ours were analyzed. The average age at treatment was 68.7 years of age ranging from 51 to 83 years with 90.9% of patients being female. 18.2% of patients had symptoms immediately after trauma. 27.3% of patients were incorrectly diagnosed preoperatively. 90.9% of all treated Morgagni hernias were located on the right side just behind the sternum with hernia sacs. The most common contents of the hernias were the greater omentum and transverse colon. The preferred method of surgery was the trans-abdominal approach. The mortality rate of the hernias was 9.1%. Morgagni diaphragmatic hernias once diagnosed should be referred for surgical repair due to the risk of bowel perforation. The transabdominal approach or laparoscopic surgery is preferred in accurately diagnosed Morgagni diaphragmatic hernias prior to surgery.
Adult ; Colon, Transverse ; Female ; Hernia ; Hernia, Diaphragmatic* ; Humans ; Laparoscopy ; Mortality ; Omentum ; Sternum

BACKGROUND AND OBJECTIVES: MUC5AC is one of the major secretory mucin genes in the human airway epithelium. MUC5AC expression is increased by a variety of inflammatory mediators. Protopanaxadiol (PPD), one of the major active metabolites in ginseng, is known to have anti-inflammatory, antitumor and antioxidant properties. However, the effects of PPD on mucin secretion of airway epithelial cells still have not been reported. Therefore, the aim of this study is to investigate the effect of PPD on lipopolysaccharide (LPS)-induced MUC5AC expression in human airway epithelial cells. MATERIALS AND METHOD: In the mucin-producing human NCI-H292 airway epithelial cells, the effect of PPD on MUC5AC expression was investigated using reverse transcription-polymerase chain reaction and enzyme immunoassay after treated with LPS. N-acetylcysteine (NAC) as a reactive oxygen species (ROS) scavenger, and apocynin as a nicotinamide adenine dinucleotide phosphate oxidase inhibitor were used to compare the inhibitory effect of PPD on LPS-induced ROS production in human NCI-H292 cells. RESULTS: LPS significantly increased MUC5AC mRNA expression and protein production. LPS also increased ROS production. PPD inhibited LPS-induced MUC5AC mRNA expression and protein production as well as ROS production. In addition, NAC and apocynin inhibited LPS-induced MUC5AC mRNA expression and protein production. CONCLUSION: These results demonstrate that PPD inhibits LPS-induced MUC5AC expression via ROS in human airway epithelial cells and the inhibitory effect of PPD was similar to that of NAC and apocynin. These findings indicate that PPD may be a therapeutic agent for control of mucus secretion and oxidative stress in human airway epithelial cells.
Acetylcysteine ; Epithelial Cells ; Epithelium ; Humans ; Immunoenzyme Techniques ; Methods ; Mucins ; Mucus ; NADP ; Oxidative Stress ; Oxidoreductases ; Panax ; Reactive Oxygen Species ; RNA, Messenger

Background and Objectives: Peroxiredoxin (Prx) is an antioxidant enzyme involved in signaling pathway. Prx2 is the most abundant in mammalian gray matter neurons and has protective role under oxidative stress. MUC5AC and MUC5B are typical mucin genes in human airway epithelial cells. Even if free radicals play a key role in chronic respiratory inflammatory diseases, the effects of the Prx2 on mucin expression and oxidative stress are not clearly known. The purpose of this study is to investigate the effect of Prx2 on lipopolysaccharide (LPS)-induced MUC5AC/5B expression and reactive oxygen species (ROS) in human airway epithelial cells.Subjects and Method In NCI-H292 cells and human nasal epithelial cells, the effects of Prx2 on LPS-induced MUC5AC/5B expression and ROS production were investigated using reverse transcriptase-polymerase chain reaction, real-time polymerase chain reaction, enzyme linked immunosorbent assay (ELISA) and flow cytometry analysis. Results: MUC5AC, MUC5B mRNA expression and protein production were increased by LPS. ROS production was also increased by LPS. Prx2 suppressed the LPS-induced MUC5AC mRNA expression and protein production as well as ROS production. However, Prx2 did not inhibit MUC5B mRNA expression and protein production. N-acetylcysteine, diphenyleneiodonium, and apocynin also inhibited LPS-induced ROS production. Conclusion These results may show that Prx2 suppresses LPS-induced MUC5AC expression via ROS in human airway epithelial cells.

BACKGROUND AND OBJECTIVES: MUC5AC is one of the major secretory mucin genes in the human airway epithelium. MUC5AC expression is increased by a variety of inflammatory mediators. Protopanaxadiol (PPD), one of the major active metabolites in ginseng, is known to have anti-inflammatory, antitumor and antioxidant properties. However, the effects of PPD on mucin secretion of airway epithelial cells still have not been reported. Therefore, the aim of this study is to investigate the effect of PPD on lipopolysaccharide (LPS)-induced MUC5AC expression in human airway epithelial cells. MATERIALS AND METHOD: In the mucin-producing human NCI-H292 airway epithelial cells, the effect of PPD on MUC5AC expression was investigated using reverse transcription-polymerase chain reaction and enzyme immunoassay after treated with LPS. N-acetylcysteine (NAC) as a reactive oxygen species (ROS) scavenger, and apocynin as a nicotinamide adenine dinucleotide phosphate oxidase inhibitor were used to compare the inhibitory effect of PPD on LPS-induced ROS production in human NCI-H292 cells. RESULTS: LPS significantly increased MUC5AC mRNA expression and protein production. LPS also increased ROS production. PPD inhibited LPS-induced MUC5AC mRNA expression and protein production as well as ROS production. In addition, NAC and apocynin inhibited LPS-induced MUC5AC mRNA expression and protein production. CONCLUSION These results demonstrate that PPD inhibits LPS-induced MUC5AC expression via ROS in human airway epithelial cells and the inhibitory effect of PPD was similar to that of NAC and apocynin. These findings indicate that PPD may be a therapeutic agent for control of mucus secretion and oxidative stress in human airway epithelial cells.

Background: and Purpose: The aim of this study was to evaluate the structural and functional connectivities of brain network using graph theoretical analysis in neurologically asymptomatic patients with end-stage renal disease (ESRD). We further investigated the prevalence of cognitive impairment (CI) in ESRD patients and analyzed the association between network measures of brain connectivity and cognitive function. Methods: We prospectively enrolled 40 neurologically asymptomatic ESRD patients, 40 healthy controls, and 20 disease controls. All of the subjects underwent diffusion-tensor imaging (DTI) and resting-state functional magnetic resonance imaging (rs-fMRI). We calculated measures of structural and functional connectivities based on DTI and rs-fMRI, respectively, and investigated differences therein between the ESRD patients and the healthy controls. We assessed cognitive function in the ESRD patients using the Korean version of the Consortium to Establish a Registry for Alzheimer’s Disease neuropsychological battery. Results: The ESRD patients exhibited decreased global structural and functional brain connectivities, as well as alterations of network hubs compared to the healthy controls and disease controls. About 70% of the ESRD patients had CI. Moreover, ESRD patients without CI exhibited decreased global connectivity and alterations of network hubs. Furthermore, there was a significant positive association between measures of brain connectivity and cognitive function. Conclusions We found that ESRD patients exhibited decreased structural and functional brain connectivities, and that there was a significant association between brain connectivity and cognitive function. These alterations in the brain network may contribute to the pathophysiological mechanism of CI in ESRD patients.

BACKGROUND: Congenital adrenal hyperplasia (CAH) results from an inherited defect in enzymatic steps required to synthesize cortisol from cholesterol. 21-hydroxylase deficiency accounts for 95% cases of CAH. It appears that the frequency and the type of the responsible mutations differ according to the ethnic background and the type of mutation can predict the clinical outcomes such as salt losing type (SL), simple virilizing type (SV) and non-classic type (NC). METHODS: We have analyzed CYP21 genes in 55 Korean cases (110 chromosomes) of CAH by Southern blotting, PCR-dot hybridization and PCR amplification-created restriction site method. The patients include 43 cases of SL and 12 of SV. None of the NC was found. RESULTS: We found the mutations in 94% (103/110) of the examined chromosomes. A total of 10 types of mutations were discovered. The mutations include aberrant splicing of intron 2 (i2, 35%), CYP21 gene deletion (32%) and I172N (11%) in order. When the relationship between the clinical types and genotypes were correlated, most of the SL patients have either i2 (42%) or CYP21 gene deletion (41%), while SV patients have I172N (33%) or P30L (21%). The parents' mutation was investigated in 20 cases. In 4 families, one of the parents was not the obligatory heterozygote carrier i.e. did not have a mutation. The results suggest the high incidence of de novo mutation. CONCLUSION: We have identified the frequency of mutations of the CYP21 in Korean AGS patients. Our results shows that the clinical type of AGS can be predicted from the genotypes of CYP21. Also the high incidence of de novo mutation of CYP21 confirmed the genetic instability of major histocompatibility III region where the CYP21 is located.
Adrenal Hyperplasia, Congenital ; Adrenogenital Syndrome* ; Blotting, Southern ; Cholesterol ; Gene Deletion ; Genotype* ; Heterozygote ; Histocompatibility ; Humans ; Hydrocortisone ; Incidence ; Introns ; Parents ; Phenotype* ; Polymerase Chain Reaction ; Steroid 21-Hydroxylase*