• Title/Summary/Keyword: Kidney tubules

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CUBN mutation: a benign genetic cause of proteinuria?

  • Hyun Kyung Lee
    • Childhood Kidney Diseases
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    • v.27 no.1
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    • pp.19-25
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    • 2023
  • Proteinuria is an important risk factor for renal and cardiovascular disease. It is associated with a risk for glomerulonephritis, chronic kidney disease, and end-stage renal disease. Therefore, if persistent proteinuria is detected, kidney biopsy is considered to diagnose and treat the underlying disease. Recently, variants in the cubilin (CUBN) gene, which is associated with albuminuria, have been reported. This gene encodes cubilin, a membrane glycoprotein receptor expressed in the renal proximal tubules. Cubilin is a component of the megalin and cubilin-amnionless complex that mediates albumin reabsorption into the proximal tubules through endocytosis. A defect in cubilin leads to a reduction in albumin reuptake, resulting in albumin-dominant proteinuria. Although numerous controversies exist, several reports suggest that cubilin defects lead to proteinuria with a high portion of albuminuria but may not impair renal filtration function. If albuminuria due to reduced cubilin function is confirmed as a benign condition, we can consider using genetic studies to detect CUBN mutations in patients with proteinuria and they may not require any treatment or kidney biopsy. Here, we review recent papers on CUBN mutations and discuss the prognosis and management of individuals with this mutation.

Glycoconjugates Properties of the Kidney in the Hibernating Chipmunks (동면 중 한국산 다람쥐 신장의 복합당질 변화)

  • Gil, Young-Gi
    • Journal of Life Science
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    • v.18 no.9
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    • pp.1239-1243
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    • 2008
  • Though relatively little is known of the physiology involved, there is reduced kidney activity during hibernation. In this experimental study, male Korean chipmunks (Tamias sibiricus barberi) were maintained in cold conditions ($6^{\circ}C$) for 9 months, in an attempt to mimic conditions occurring during seasonal hibernation. The examination was made to determine changes of glycoconjugates on the kidney after hibernation by lectin histochemistry. The changes of lectin affinities in the kidney categorize into three groups: a) increase ones only in the early hibernation stage b) increase ones during hibernation and c) decrease ones during hibernation. The transient increase of Con A affinity in the proximal convoluted tubules were demonstrated only in the early cold-treated stage, and PNA and Con A in the distal convoluted tubules and DBA and sWGA in the collecting tubules. SBA affinity tended to increase with hibernation in the proximal convoluted tubules, but sWGA affinities were significantly decreased in the all tubule examined with hibernation. The present results suggest that the glycosylation pattern of the kidney undergoes profound changes during hibernation, and is probably associated with transiently reduced renal function.

The Effects of Kami-bangpungtongseong-san Extracts on the Mouse Liver and Kidney Toxicated by Mercuric Chloride Subcutaneous Injection (가미방풍통성산(加味防風通聖散)이 수은의 피하 주입으로 중독된 마우스의 간 및 신기능에 미치는 영향)

  • Yang, Joon-Hyoung;Hong, Seong-Ug
    • The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
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    • v.23 no.2
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    • pp.1-12
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    • 2010
  • Objective : For this study, mice on mercurial toxication were given mercuric subcutaneous injection to their abdomen factitiously. After delivering Kami- bangpungtongseong-san(KBT) extracts to the mice by oral administration, we observed changes from liver and kidney of mice. Method : The BALB/c mice were distributed into three groups: No treated group(Normal group), Mercuric chloride subcutaneous injection group(Control group), Kami-bangpungtongseong-san-treated group (Sample group). KBT Extracts were delivered orally in 7 days. We observed involution of liver, necrosis of liver and cell plate loss of liver, lipid peroxidation CYP1A1 expression. We observed involution of proximal convoluted tubules, hypertrophy of Bowman's capsule, periodic acid-Schiff(PAS)'s positive reaction of proximal convoluted tubules, heat shock protein(HSP)700's positive reaction in glomerulus. For the charting the results, image analysis was taken. The result of image analysis was verified significance by Sigmaplot 2000(P<0.05). Result : The mice' liver on mercurial toxication were relieved involution of liver, necrosis of liver, and cell plate loss of liver and also declined lipid peroxidation and CYP1A1 expression. The mice' kidney on mercurial toxication were relieved involution of proximal convoluted tubules, hypertrophy of Bowman's capsule and increasing PAS's positive reaction of proximal convoluted tubules. On the other hand it was declined HSP700's positive reaction in glomerulus. Conclusion : According to the result of study, we think that we can expect to the effect of KBT extracts' therapeutic action to tissue injuries of the mice' liver and kidney on acute mercurial toxication.

Protection Effect of Natual Matter and Radiation Damage on Kidney Tissue (신장 조직의 방사선 손상과 천연물질의 방어기전 연구)

  • Ji, Tae-Jeong
    • The Journal of the Korea Contents Association
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    • v.9 no.12
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    • pp.376-384
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    • 2009
  • This research has microstructure observation to find tissue damage mechanism and radio-protection effect on mouse kidney tissue. The result observation of a Light Microscope(LM); The kidney tissue after 5Gy irradiation observed a glomerulus atrophy, also crack distance to base membrane of a convoluted tubules. The kidney tissue after 10Gy irradiation observed out flow cytoplasm to membrane break of a convoluted tubules. The result observation of a Transmission Electron Microscope(TEM); The kidney tissue of after 5Gy irradiation has to breaking a inside cristae and membrane of mitochondria, also show definite damage of nucleus membrane. 10Gy irradiation has all the more damage a base membrane and thickness of lysosome. However, Propolis eating groups observed normal to nucleus membrane and small body of intracellular. therefore We considered "Propolis" as make radio protection function to kidney tissue of the greater part.

Pathological Observations of Polycystic Kidney in a Korean Native Calf (한우(韓牛)에 발생(發生)한 Polycystic Kidney)

  • Kwak, Soo Dong;Lee, Cha Soo
    • Korean Journal of Veterinary Research
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    • v.19 no.1
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    • pp.27-32
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    • 1979
  • This paper dealt with the macroscopical and histopathological observations on the polycystic kidney occurred in a Korean native calf aged about a year. The results summarized are as follows: 1. In macroscopical findings, numerous cysts in the bilateral kidneys were seen under the renal capsule, and the cysts were various in size and clear or cloudy in their contents. The cysts in the inner area of the renal cortex were smaller than those of the outer area of the renal cortex in size and in number. 2. In microscopical findings, marked dilatations of Bowman's spaces and convoluted tubules were prominant feature. Numerous cysts with or without eosinophilic materials were contained atrophic glomerulus. prolferation of fibrous connective tissue, atrophy of convoluted tubules were also observed. Inner walls of the cysts were surrounded by cuboidal cells, sguamous cells or fibrous connective tissue. 3. This case was regarded as congenital polycystic kidney belonged to type III of Osathanondh and Potter.

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Tubulopathy: the clinical and genetic approach in diagnosis

  • Jinwoon Joung;Heeyeon Cho
    • Childhood Kidney Diseases
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    • v.27 no.1
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    • pp.11-18
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    • 2023
  • Remarkable advances in genetic diagnosis expanded our knowledge about inherited tubulopathies and other genetic kidney diseases. This review suggests a simple categorization of inherited tubular disease, clarifies the concept of autosomal dominant tubulointerstitial kidney disease (ADTKD), and introduces novel therapies developed for tubulopathies. Facing patients with suspicious tubular disorders, clinicians should first evaluate the status of volume and acid-base. This step helps the clinicians to localize the affected segment and to confirm genetic diagnosis. ADTKD is a recently characterized disease entity involving tubules. The known causative genes are UMOD, MUC1, REN, and HNF1β. Still, only half of ADTKD patients show mutations for these four identified genes. Whole exome sequencing is a suitable diagnostic tool for tubulopathies, especially for ADTKD. Genetic approaches to treat tubulopathies have progressed recently. Despite the practical obstacles, novel therapies targeting inherited tubulopathies are currently in development.

Receptor Subtypes for Endothelin in the Kidney of the Freshwater Turtle (Amyda japonica)

  • Kim, Sung-Zoo
    • Animal cells and systems
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    • v.4 no.1
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    • pp.63-70
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    • 2000
  • The distribution of receptor subtypes for endothelin (ET) in the kidney of the freshwater turtle, Amyda japonica, was examined by quantitative in vitro receptor autoradiography using iodinatd mammalian type ET-1 ($^125$/I-ET-1)as a radiolabeled ligand. Specific $^125$/I-ET-1 bindings were localized to renal tubules, renal arteries and ureter with binding densities of 111.21 $\pm$ 19.14, 182.13$\pm$10.57 and 219.46$\pm$12.83 amol/$mm^2$. respectively. Binding dissociation constants in renal tubules, renal arteries and ureter were 1.05 $\pm$ 0.63, 2.03 $\pm$0.56 and 1.70$\pm$0.47nM, respectively. Receptor subtypes for ET in the kidney were characterized by competition with BQ 123 and BQ 788 as specific antagonists for ET receptors, type A (ET$_A$ ), and type B (ET$_B$) subtypes, respectively. Specific $^125$/I-ET-1 bindings in renal arteries and ureter were potently inhibited by BQ 123 in a dose-dependent manner, whereas BQ 788 was not in competing for specific $^125$/I-ET-1 bindings in this structure. However, specific $^125$/I-ET-1 bindings in renal tubules were inhibited more potently by BQ 788. Therefore, these results indicate that specific ET receptors are localized in renal tubules, renal arteries and the ureter of the freshwater turtle. Results also suggest that the predominant ET receptor subtypes are like the ETA receptor in renal arteries and ureter, and like the ET/$_A$ receptor in the renal tubule.

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Detection of Mercury in Kidney, Liver, Spleen and Cerebellum of the Mouse by Autometallography (오토메탈로그라피에 의한 마우스의 신장, 간장, 비장, 및 소뇌에 축적된 수은의 검출)

  • 조현욱;김명훈;황규영;이성태
    • Toxicological Research
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    • v.13 no.4
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    • pp.401-408
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    • 1997
  • Adult male ICR mice were exposed to methylmercuric chloride (CH$_3$HgCI) through drinking water for 80 days. The distribution of mercury in the kidney, liver, spleen and cerebellum of the mouse was examined according to a autometallographic silver-enhancement technique based on a physical development process which renders mercury deposit visible. Grains of mercury traces were located in the proximal convoluted tubules. Lesser staining of the grains was seen in the collecting tubules of medulla. The glomerular basement membrane was void. In the liver, mercury accumulations were present primarily in the hepatocytes around portal area containing interlobular bile duct, artery and portal vein. Also grains of mercury traces were accumulated in the white pulp of the spleen and Purkinje cell layer of the cerebellum.

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Effect of Verapamil on Renal Function in Dog (Verapamil이 개의 신장기능에 미치는 영향)

  • 고석태;허영근
    • YAKHAK HOEJI
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    • v.35 no.2
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    • pp.85-98
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    • 1991
  • Verapamil, $Ca^{2+}$-channel blocker, when given into vein or into carotid artery, produced the decrease of urine flow accompanied with the decreased amounts of Na$^{+}$ and $K^{+}$ excreted in urine ($E_{Na}, E_{K}$) and with the decreased clearances of free water (C$_{H_2O}$) and osmolar substance (C$_{osm}$), and then increased reabsorption of Na$^{+}$ and $K^{+}$ in renal tubules (R$_{Na}$, R$_{N}$), glomeruler filtration rate (GFR) and renal plasma flow (RPF) were inhibited when verapamil was given into carotid artery, but were only tendency of reduction when given intravenously. Verapamil, when infused into a renal artery, exhibited diuresis accompanied with the increased GER, RPF, E$_{Na}$ and E$_{K}$, with the decreased filtration fraction (FF) in only infused kidney. At the same time, $C_{H_2O}$ was not changed, R$_{Na}$ and R$_{K}$ were reduced. Antidiuretic action by verapamil administered into vein or into carotid artery in normal kidney was reversed to diuretic action in denervated kidney. At this time, parameters of renal function exhibited the opposite phenomena compared to that elicited by verapamil in normal kidney, wherease renal denervation did not influence the action of verapamil infused into a renal artery. Above results suggest that verapamil produce both antidiuresis through nervous system centrally, not endogenous substances and diuresis by direct action in the kidney. Diurectic action are caused by hemodynamic improvement through dilatioon of vas efferense and by greatly inhibited reabsorption of electrolytes in distal tubules.

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Effects of Glibenclamide, an ATP-dependent $K^+$ Channel Blocker, on Renal Function in Dog (ATP 의존성 $K^+$ Channel 차단작용이 있는 Glibenclamide가 개의 신장기능에 미치는 영향)

  • 고석태;임광남
    • Biomolecules & Therapeutics
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    • v.7 no.3
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    • pp.249-256
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    • 1999
  • Glibenclamide(GLY)(1.0 and 3.0 mg/kg), an ATP-dependent $K^+$ channel blocker, when given into the vein in dogs, produced the diuretic action accompanied with the increase of osmolar clearance($C_{osm}$), urinary excretion of $Na^+$ and $K^+$ ($E_{Na}$, $E_K$), and with the decrease in reabsorption rates for $Na^+$ and $K^+$ in renal tubules ($R_{Na}$, $R_K$), and then ratios of $K^+$ against $Na^+$($K^+$/$Na^+$) were decreased. GLY did not affect mean arterial pressure at any doses used. At a low dose(0.1 mg/kg), GLY injected into a renal artery brought about the diurectic action in both experimental and control kidney, however at a higher dose(0.3 mg/kg), GLY appeared significant diuretic action in the control kidney, but not in experimental kidney and the decrease of glomerular filtration rates(GFR), renal plasma flow(RPF), $E_K$, and the increase in $E_{Na}$. In the control kidney, these changes in renal function exhibited the same aspect as shown in intravenous experiments. In experiments given into carotid artery of GLY(0.5 and 1.5 mg/kg), changes in all renal function included the increase in urine volume were the same pattern as shown in intravenous experiments. The above results suggest that glibenclamide produces diuretic action through central function and the action site of the GLY in kidney is the renal distal tubules in dogs.

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