• Biomolecules & Therapeutics
• /
• v.29 no.3
• /
• pp.311-320
• /
• 2021

Accumulation of reactive oxygen species (ROS) is associated with the development of various diseases. However, the molecular mechanisms underlying oxidative stress that lead to such diseases like autosomal dominant polycystic kidney disease (ADPKD) remain unclear. Here, we observed that oxidative stress markers were increased in Pkd1f/f:HoxB7-Cre mice. Forkhead transcription factors of the O class (FOXOs) are known key regulators of the oxidative stress response, which have been observed with the expression of FoxO3a in an ADPKD mouse model in the present study. An integrated analysis of two datasets for differentially expressed miRNA, such as miRNA sequencing analysis of Pkd1 conditional knockout mice and microarray analysis of samples from ADPKD patients, showed that miR-132-3p was a key regulator of FOXO3a in ADPKD. miR-132-3p was significantly upregulated in ADPKD which directly targeted FOXO3 in both mouse and human cell lines. Interestingly, the mitochondrial gene Gatm was downregulated in ADPKD which led to a decreased inhibition of Foxo3. Overexpression of miR-132-3p coupled with knockdown of Foxo3 and Gatm increased ROS and accelerated cyst formation in 3D culture. This study reveals a novel mechanism involving miR-132-3p, Foxo3, and Gatm that is associated with the oxidative stress that occurs during cystogenesis in ADPKD.

• Clinical and Experimental Pediatrics
• /
• v.52 no.4
• /
• pp.464-470
• /
• 2009

Purpose : This study assessed the incidence and outcome of congenital anomalies of the kidney and urinary tract (CAKUT) detected by prenatal ultrasonography Methods : There were 906 cases of CAKUT detected by prenatal ultrasonography and postnatally confirmed at the Asan Medical Center from October 1989 to October 2007. We investigated the incidence and outcome of these cases by reviewing medical records retrospectively. Results : The order of incidence was hydronephrosis, multicystic dysplastic kidney (MCDK), duplex kidney, vesico-ureteral reflux (VUR), single kidney, hydroureteronephrosis, ectopic kidney, polycystic kidney, ureterocele, and posterior urethral valve (PUV). There were 520 cases (57.4%) of hydronephrosis, and 20% of these needed an operation due to significant obstruction. MCDK was associated with other CAKUT in 25.4% of all cases. Approximately 57.9% of duplex kidney cases needed surgical treatment due to ureterocele and VUR. VUR had a male: female ratio of 10:1. Two out of seven cases of autosomal recessive polycystic kidney had progressed to chronic renal failure. Patients with PUV were relatively uncommon, and one out of nine cases progressed to end-stage renal disease. Conclusion : CAKUTs detected by prenatal ultrasonography were composed of various anomalies, and almost all of them had a good outcome without any intervention. However, in some cases, recurrent urinary tract infection or renal failure occurred, especially in bilateral cases. For further management, a long-term multicenter study is needed to investigate the precise incidence and outcome of each anomaly in the general population.

• The Journal of Korean Medicine
• /
• v.41 no.1
• /
• pp.84-92
• /
• 2020

Objectives: To observe the effects on liver and kidney of gynecology patients' during long term Korean Medicine. Methods: This research was conducted as a panel study on 29 female gynecology patients at Korean medicine clinic and compares their conditions before, 3 months after, and 6 months after Intake of Korean medicine. This analysis was conducted to frequency(%), repeated measure ANOVA and significantly statistics was p<0.05, confidence interval. Results: The most common conditions among the subjects were polycystic ovary syndrome, endometriosis, adenomyosis, and uterine myoma. Except in a few instances, liver functions (AST, ALT, γ-GTP) were within the normal range before and after the intake of Korean medicine. Further, while there was a slight change in liver functions when three months and six months after the intake, but there was no statistical significance (p>0.05). As for the kidney functions (BUN, Creatinine, BUN/Creatinine), they were within the normal range before and after the intake of Korean medicine. Further, while there was a slight change in kidney functions three months and six months after the intake, there was no statistical significance(p>0.05) either. Conclusions: Based on these results, it can be concluded that Korean medicine does not have a negative impact on the gynecology patients' liver and kidney.

• Proceedings of the Zoological Society Korea Conference
• /
• 1998.04a
• /
• pp.60.1-60
• /
• 1998

No Abstract, See Full Text

• BMB Reports
• /
• v.53 no.7
• /
• pp.367-372
• /
• 2020

Cell cycle-related kinase (CCRK) has a conserved role in ciliogenesis, and Ccrk defects in mice lead to developmental defects, including exencephaly, preaxial polydactyly, skeletal abnormalities, retinal degeneration, and polycystic kidney. Here, we found that Ccrk is highly expressed in mouse trachea and bronchioles. Ccrk mutants exhibited pulmonary hypoplasia and abnormal branching morphogenesis in respiratory organ development. Furthermore, we demonstrated that Ccrk mutant lungs exhibit not only impaired branching morphogenesis but also a significant sacculation deficiency in alveoli associated with reduced epithelial progenitor cell proliferation. In pseudoglandular stages, Ccrk mutant lungs showed a downregulation of Hedgehog (Hh) signaling and defects in cilia morphology and frequency during progenitor-cell proliferation. Interestingly, we observed that activation of the Hh signaling pathway by small-molecule smoothened agonist (SAG) partially rescued bud morphology during branch bifurcation in explants from Ccrk mutant lungs. Therefore, CCRK properly regulates respiratory airway architecture in part through Hh-signal transduction and ciliogenesis.

• Clinical and Experimental Pediatrics
• /
• v.57 no.1
• /
• pp.1-18
• /
• 2014

Channelopathies are a heterogeneous group of disorders resulting from the dysfunction of ion channels located in the membranes of all cells and many cellular organelles. These include diseases of the nervous system (e.g., generalized epilepsy with febrile seizures plus, familial hemiplegic migraine, episodic ataxia, and hyperkalemic and hypokalemic periodic paralysis), the cardiovascular system (e.g., long QT syndrome, short QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia), the respiratory system (e.g., cystic fibrosis), the endocrine system (e.g., neonatal diabetes mellitus, familial hyperinsulinemic hypoglycemia, thyrotoxic hypokalemic periodic paralysis, and familial hyperaldosteronism), the urinary system (e.g., Bartter syndrome, nephrogenic diabetes insipidus, autosomal-dominant polycystic kidney disease, and hypomagnesemia with secondary hypocalcemia), and the immune system (e.g., myasthenia gravis, neuromyelitis optica, Isaac syndrome, and anti-NMDA [N-methyl-D-aspartate] receptor encephalitis). The field of channelopathies is expanding rapidly, as is the utility of molecular-genetic and electrophysiological studies. This review provides a brief overview and update of channelopathies, with a focus on recent advances in the pathophysiological mechanisms that may help clinicians better understand, diagnose, and develop treatments for these diseases.

• The Journal of Internal Korean Medicine
• /
• v.25 no.4
• /
• pp.192-199
• /
• 2004

Objective : This study was designed to evaluate the extent of effectivness of no mind-therapy (Korean traditional Qigong) on ADPKD. Method : ADPKD patient were observed for one year, given no Western Treatments, and treated only Josik(調息), Joki(調氣)(Korean traditional therapy). Results : 1. The general condition of patient improved and there were specific symtoms that ceased. 2. Hypertention patient showed a lowering of blood pressure. Conclusion : These results suggest a role for no mind-therapy in treatement of ADPKD.

• Journal of Physiology & Pathology in Korean Medicine
• /
• v.27 no.2
• /
• pp.153-166
• /
• 2013

Yang deficiency pattern is a representative syndrome differentiation. This article is a study on to which categories of modern diseases yang deficiency is assigned by reference to modern clinical papers and the meaning of yang deficiency interpreted with a perspective of Korean Medicine and a modern perspective. Yang deficiency, yang qi deficiency, lack of yang qi and yang qi debilitation are the words found in "Nei Ching" and yang qi can be interpreted as something to warm, drive and arouse. Zhangzhongjing considered recovery or loss of Yang as the key to life in "Shanghanlun". Danxi proposed "Yang being liable to hyperactivity, Yin being insufficient" and emphasized pathological ministerial fire of Yang exuberance rather than physiological ministerial fire of Yang deficiency. Zhangjingyue proposed "Yang not being in excess, Yin being often deficient" and understood growth and decline of yin qi are all led by yang qi and put emphasis on true yin in addition to yang qi. Diseases of yang deficiency pattern are related with decline of metabolic level, hypofunction of internal secretion, disorder of immune function, disorder of automatic nerve system, sympathetic nerve inhibition, metabolic disorder of microelements, increase of cGMP, change of microcirculation, low speed of blood stream, kidney malfunction. Diseases related with kidney are sterility, polycystic ovary syndrome, spinal stenosis, edema, renal failure, IgA nephropathy, erectile dysfunction, nephritis, prostatitis, benign prostatic hyperplasia, decrease of adrenal cortical hormone by nephrotic syndrome, myelodysplastic syndrome. Disease related with heart are heart failure, arrhythmia, cardiomyopathy, atherosclerosis heart disease, hypertension, hyperlipidemia, pulmonary heart disease. Diseases related with spleen are irritable bowel syndrome, ulcerative colitis. Diseases related with liver are hypothyroidism, liver cirrhosis ascites, hepatitis B, chronic hepatitis, hepatic diabetes. Diseases related with lung are allergic rhinitis, cough variant asthma, bronchial asthma, pulmonary emphysema. And diabetes mellitus, metabolic syndrome, aplastic anemia, headache, encephalatrophy, Alzheimer's disease are also related with yang deficiency.

• Asian-Australasian Journal of Animal Sciences
• /
• v.21 no.12
• /
• pp.1794-1799
• /
• 2008

This preliminary feeding trial was conducted to study the effects of different dietary selenium (Se) levels on growth performance and toxicity in juvenile black seabream, Acanthopagrus schlegeli (Bleeker). Fish averaging $7.0{\pm}0.1g$ ($mean{\pm}SD$) were fed one of the five semi-purified diets containing 0.21, 0.30, 0.52, 1.29 and 12.3 mg sodium selenite ($Na_2SeO_3$)/kg diet (Se 0.21, Se 0.30, Se 0.52, Se 1.29 or Se 12.3) for 15 weeks. After the feeding trial, weight gain (WG), feed efficiency (FE), specific growth rate (SGR) and protein efficiency ratio (PER) of fish fed Se 0.21, Se 0.30, Se 0.52 and Se 1.29 diets were not significantly different, however fish fed Se 12.3 diet showed significantly lower WG, FE, SGR and PER than those of fish fed the other diets (p<0.05). Fish fed Se 0.21, Se 0.30, Se 0.52, Se 1.29 and Se 12.3 diets showed no significant differences in hematocrit (PCV), hemoglobin (Hb) and red blood cells (RBC), however fish fed Se 12.3 diet showed lower values of PCV, Hb and RBC than those of fish fed the other diets. Histopathological lesions such as tubular necrosis and polycystic dilation of tubules in the kidney tissues were observed in fish fed Se 12.3 diet. Se was accumulated in a dose-dependent manner in the liver, kidney, muscle and gill tissues. Based on the results of this preliminary feeding trial, a dietary Se level of 0.21 mg $Na_2SeO_3/kg$ diet could be optimal for proper growth performances, and a dietary Se level of 12.3 mg $Na_2SeO_3/kg$ diet may ultimately be toxic to juvenile black seabream, Acanthopagrus schlegeli.

• Journal of Genetic Medicine
• /
• v.11 no.1
• /
• pp.31-35
• /
• 2014

Bardet-Biedl syndrome (BBS) is a rare ciliopathy generally inherited with an autosomal recessive pattern. BBS is characterized by 6 primary features namely retinal dystrophy, obesity, postaxial polydactyly, renal dysfunction, learning difficulties, and hypogonadism and a wide range of secondary features. To date, mutations in 16 genes have been identified as causative factors for BBS. Among them, the BBS1 and BBS10 genes are major disease-causing genes, and each of these gene mutations presents in more than 20% of all BBS patients. Genotype-phenotype correlations have not been observed in BBS, and there can be phenotypic overlap between BBS and other ciliopathies. In Korea, no molecular, genetically confirmed case of BBS has been reported to date. Herein, we describe the case of the first Korean siblings with BBS resulting from 2 BBS10 gene mutations who showed typical clinical phenotypes, including retinal dystrophy, obesity, intellectual disability, cystic tubular disease, and postaxial polydactyly.