• 한국동물학회:학술대회논문집
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• 한국동물학회 2001년도 한국생물과학협회 학술발표대회
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• pp.106.1-106
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• 2001

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• BMB Reports
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• 제46권7호
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• pp.338-345
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• 2013

microRNAs (miRNAs) are small non-coding RNAs that regulate gene expression by targeting the 3'-untranslated region of multiple target genes. Pathogenesis results from defects in several gene sets; therefore, disease progression could be prevented using miRNAs targeting multiple genes. Moreover, recent studies suggest that miRNAs reflect the stage of the specific disease, such as carcinogenesis. Cystic diseases, including polycystic kidney disease, polycystic liver disease, pancreatic cystic disease, and ovarian cystic disease, have common processes of cyst formation in the specific organ. Specifically, epithelial cells initiate abnormal cell proliferation and apoptosis as a result of alterations to key genes. Cysts are caused by fluid accumulation in the lumen. However, the molecular mechanisms underlying cyst formation and progression remain unclear. This review aims to introduce the key miRNAs related to cyst formation, and we suggest that miRNAs could be useful biomarkers and potential therapeutic targets in several cystic diseases.

• 대한한방내과학회지
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• 제42권3호
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• pp.225-238
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• 2021

Objective: This research reviews and investigates the trends in recent clinical studies of polycystic kidney disease (PKD) in China. Method: We searched for clinical studies discussing Oriental medicine-based treatments for PKD in the China National Knowledge Infrastructure (CNKI) database. Thirteen clinical articles published from 2001 to 2019 were analyzed. The search focused on the authors, publication year, type of study, purposes of study, method and duration of treatment, evaluation criteria, and results of the selected articles. Results: Of the articles from the database, 9 case series and 4 randomized controlled trials (RCTs) were analyzed. Ten articles used herbal medicine; 4 used herbal medicine for external use. Gamigyejibokryeong-hwan was the most common herbal prescription. The most frequently used herb was Polia Sclerotium (茯苓), and Cnidii Rhizoma (川芎) was employed in all the external uses. All 13 studies confirmed the efficacy of Oriental medicine treatments. Conclusion: 1. Scientifically designed and more varied clinical studies are required to develop treatments for PKD. 2. The current study could be used as basic data in future clinical studies on treatment and further studies of PKD.

• 한국임상수의학회지
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• 제27권6호
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• pp.726-728
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• 2010

상염색체 우성 다낭성 신병은 페르시안과 페르시안에서 유래된 품종에서 다발하는 질환으로, 스코티쉬 폴드 고양이에서도 간혹 보고된 바가 있다. 5살령의 수컷 스코티쉬 폴드 고양이가 기본적인 혈액 검사와 복부 초음파 등을 통해 다낭성 신병으로 진단 받은 후 3.5 개월 만에 폐사하였다. 이 고양이는 동배 암컷 고양이를 비롯한 3 마리의 암컷 고양이와 교배하여 14마리의 새끼고양이가 있었으며 연령대는 3개월령에서 8년령으로 다양하였다. 상염색체 우성 다낭성 신병인지를 확인하기 위해 변이된 PKD1 유전자에 대한 유전자 검사가 이루어졌다. 또한 복부 초음파를 통해 신장의 낭성 구조물을 확인하는 검사도 이루어졌다. 총 19마리 (수컷: 13마리, 암컷: 6 마리) 에 대한 검사가 이루어 졌으며 연령대는 3개월에서 8년령 사이였다. 검사 결과, 19마리 모두에서 유전자 검사와 초음파 검사 결과가 일치하였고, 이 중 8마리가 상염색체 우성 다낭성 신병으로 진단되었다. 아직까지 한국에서는 고양이의 상염색체 우성 다낭성 신병은 보고 된바 없으며, 본 조사는 가족 단위의 스코티쉬 폴드 고양이에 발생한 상염색체 우성 다낭성 신병에 대한 첫 보고이다.

• 보험의학회지
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• 제2권1호
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• pp.218-232
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• 1985

Congenital hereditary disease is in devided into Infantile type and Adult type, Adult type is hidden for many years and keeps normal renal function till middle age. Cyst is stimultaneously made in both sides and becomes lowered in renal function in 30's to 40's. Infantile type is generally born with the big kidneys, renal failure, undergrowth of intrahepatic bile duct. Both infantile and childhood type have ureteral dilatation and portal hypertension In infantile type, it is mostly developed into renal failure, but generally faces death as a result of hepatic disease. The reason of death is that an abnormal condition of recessive autosome affects the liver and kidneys. While the incidence of infantile type is rare as $0.017{\sim}0.07%$ and it is autosomal recessive heredity, Adult type can rarely exist in infantile period. Though it exists in middle period, 50% of patients can live for 2-4 years after the first symptom incidence and 25% can less than 2 years. It is hard to cure completely in medicine and surgery. Three difficulties in familial incidence are comparative decrease of the donor who have no affection on renal transplantation. For another consideration it is to show the family history for several generations. We, the Med. Dept. of Dae Han Kyouk Life Insurance Co. Ltd., used the ultrasonic apparatus in diagnosing the one case of adult type bilateral polycystic kidney and then doubted the family history. As a result of inspecting the family we experienced bilateral polycystic kidney from 3 persons out of 4 who can be inspected. The results are as follows: 1) We could confirm the polycystic kidney from 3 persons out of 4(75%). 2) Then when they came for check up, chief complaint was the pain in all 3 cases(100%). 3) Accompanying disease was hypertension in 2 cases(67%). 4) In early disease incidence, we couldn't observe the specific change in pathological opinion. 5) All 3 cases are not accompanied with cystic lesion in liver, spleen, pancreas.

• BMB Reports
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• 제37권5호
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• pp.612-617
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• 2004

Autosomal dominant polycystic kidney disease (ADPKD) is a common genetic disorder characterized by the formation of fluid-filled cysts in the kidney and progressive renal failure. Other manifestations of ADPKD include the formation of cysts in other organs (liver, pancreas, and spleen), hypertension, cardiac defects, and cerebral aneurysms. The loss of function of the polycystin -1 and -2 results in the formation of epithelium-lined cysts, a process that depends on initial epithelial proliferation. cDNA microarrays powerfully monitor gene expression and have led to the discoveries of pathways regulating complex biological processes. We undertook to profile the gene expression patterns of epithelial cells derived from the cysts of ADPKD patients using the cDNA microarray technique. Candidate genes that were differently expressed in cyst tissues were identified. 19 genes were up-regulated, and 6 down-regulated. Semi-quantitative RT-PCR results were consistent with the microarray findings. To distinguish between normal and epithelial cells, we used the hierarchical method. The results obtained may provide a molecular basis for understanding the biological meaning of cytogenesis.

• The Korean Journal of Physiology and Pharmacology
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• 제24권3호
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• pp.277-286
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• 2020

Polycystic kidney disease 2-like-1 (PKD2L1), also known as polycystin-L or TRPP3, is a non-selective cation channel that regulates intracellular calcium concentration. Calmodulin (CaM) is a calcium binding protein, consisting of N-lobe and C-lobe with two calcium binding EF-hands in each lobe. In previous study, we confirmed that CaM is associated with desensitization of PKD2L1 and that CaM N-lobe and PKD2L1 EF-hand specifically are involved. However, the CaM-binding domain (CaMBD) and its inhibitory mechanism of PKD2L1 have not been identified. In order to identify CaM-binding anchor residue of PKD2L1, single mutants of putative CaMBD and EF-hand deletion mutants were generated. The current changes of the mutants were recorded with whole-cell patch clamp. The calmidazolium (CMZ), a calmodulin inhibitor, was used under different concentrations of intracellular. Among the mutants that showed similar or higher basal currents with that of the PKD2L1 wild type, L593A showed little change in current induced by CMZ. Co-expression of L593A with CaM attenuated the inhibitory effect of PKD2L1 by CaM. In the previous study it was inferred that CaM C-lobe inhibits channels by binding to PKD2L1 at 16 nM calcium concentration and CaM N-lobe at 100 nM. Based on the results at 16 nM calcium concentration condition, this study suggests that CaM C-lobe binds to Leu-593, which can be a CaM C-lobe anchor residue, to regulate channel activity. Taken together, our results provide a model for the regulation of PKD2L1 channel activity by CaM.

• 한국임상수의학회:학술대회논문집
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• 한국임상수의학회 2009년도 춘계학술대회
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• pp.302-302
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• 2009

• Childhood Kidney Diseases
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• 제21권1호
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• pp.1-7
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• 2017

Mitogen-activated protein kinases (MAPKs) play important roles in various cellular functions including proliferation, differentiation, and apoptosis. We showed that MAPKs are developmentally regulated in the rat kidney. p38 MAPK (p38) and extracellular signal-regulated kinase (ERK) were strongly expressed in the fetal kidney, whereas c-Jun N-terminal kinase (JNK) was detected predominantly in the adult kidney. The inhibition of p38 or ERK in organ culture resulted in reduced nephron formation with or without reduced kidney size. On the other hand, persistent fetal expression pattern of MAPKs, i.e., upregulation of p38 and ERK and downregulation of JNK, was observed in the cyst epithelium of human renal dysplasia, ovine fetal obstructive uropathy, and pcy mice, a model of polycystic kidney disease. Furthermore, activated p38 and ERK induced by cyclic stretch mediated proliferation and $TGF-{\beta}1$ expression in ureteric bud cells, probably leading to cyst formation and dysplastic changes. Inhibition of ERK slowed the disease progression in pcy mice. Finally, ERK and p38 were inactivated in the early embryonic kidney subjected to maternal nutrient restriction, characterized by reduced ureteric branching and nephron number. Thus, MAPKs mediate the development of normal and diseased kidney. Their modulation may result in novel therapeutic strategies against developmental abnormalities of the kidney.

• Childhood Kidney Diseases
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• 제1권2호
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• pp.144-150
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• 1997

목적 : 낭포성 신질환은 다양한 임상증상, 조직학적 소견 및 예후를 나타내는 질환군이다. 이 질환은 종류에 따라 임상 증상이 발현되는 시기나 나타나는 신질환의 정도가 다르다. 유전성 또는 발달장애가 원인인 낭포신이 있고, 소아에서는 드물지만, 성인에서는 후천성으로 생기는 낭포신이 있다. 다양한 원인에도 불구하고 낭포형성의 과정은 비슷한 것으로 알려져 있다. 최근들어 영상진단의 발달로 본 질환의 조기 진단이 가능해졌으나 아직 그 치료는 만족스럽지 못하다. 본 연구에서는 낭포성 신질환의 분포, 빈도, 동반기형, 발견동기, 치료 및 예후에 대해서 알아보고자 한다. 방법 : 1986년 1월부터 1996년 12월까지 11년간 18세 미만의 낭포성 신질환 환아 44명을 대상으로 임상 기록지를 토대로 후향적 고찰을 시행하였다. 결과 : 1) 낭포성 신질환 환아 44명중 다발성 낭포성 이형성 신(multicystic dysplastic kidney)이 31명(71%)으로 가장 많았고, 다낭포 신(polycystic kidney)이 7명(16%)이었으며 이들 모두 상염색체 열성 유전 다낭포신(Autosomal recessive polycystic kidney disease)이었다. 단순 신낭포(simple cyst)는 5명(11%)이었으며 수질성 해면신 (medullary sponge kidney)이 1명 있었다. 2) 다발성 낭포성 이형성 신 환아 31명중 11명(35%)이 암종과의 감별 및 복부 팽만 등의 이유로 신절제술을 시행받았다. 3) 전체 낭포성 신질환 환아중 14명(32%)이 신생아 시기에 발견되어 진단만 받았을뿐 더이상 검사나 추적관찰을 하지 않았다. 4) 전 예에서 신부전의 증거는 없었다. 결론 : 본 연구결과 낭포성 신질환의 정확한 발생빈도는 알 수 없었으나 그 발생 기전과 분류 및 치료기준에 대한 지속적인 연구가 필요하다고 사료된다.ed cord 증후군에서 비뇨기계의 증상 및 비정상 검사 소견이 동반되는 경우가 흔하며 비뇨기계의 이상을 시사하는 증상이 없는 경우에도 검사상 이상이 발견되는 경우가 적지 않다. Tethered cord 증후군 환아의 평가는 신경학적 이상 유무는 물론이고 비뇨기계의 이상 여부에 대한 평가도 필히 포함되어야 할 것으로 생각된다.X> 섭취량은 배추의 경우 3.72배, 양배추의 경우 4.18배, 양상추의 경우 6.50배나 많았다. 6. 결론적으로 같은 량의 엽채류를 섭식하면서 일일 질산염 섭취량을 줄이기 위해서는 배추, 양배추, 양상추 모두 외부엽보다는 내부엽을 선택적으로 소비하는 것이 바람직함을 알 수 l있었다.었다. 6. 녹즙 종류별 ${NO_3}^-$ 함량은 당근녹즙(143ppm) < 명일엽(506ppm) < 돌미나리(669ppm) < 케일녹즙(985ppm) 순으로 많았고, Vitamin C 함량은 당근(43ppm) < 돌미나리(289ppm) < 케일(353ppm) < 명일엽(768ppm)의 순으로 높았다. 7. 일일 ${NO_3}^-$ 섭취량은 500ml의 녹즙을 마시는 경우 명일엽 253mg, 돌미나리 335mg, 케일 483mg으로 녹즙만으로도 이미 WHO/FAO의 일일 ${NO_3}^-$ 섭취허용량보다 1.16배, 1.53배, 2.21배나 초과할 수 있는 것으로 나타났다. 연약한 곤충의 방제에 효과적인 것으로 나타났다. 따라서 제조된 살충비누를 활용하면 환경친화적인 해충방제가 가능하다고 판단되었다.소변의 이상소견이 발견되어 신장 조직검사를 실시할 경우 혈청 $C_3$치의 감소 여부에 관계없이 MPGN도 진단적 고려 대상이 되어야 한다고 생각한다.신장 조직검사를 시행한 결과 진행성 경과를 취할 수 있는 막 증식성 사구체 신염과 매우 희귀한 증례인 신유전분증 등으로