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Academic literature on the topic 'Cilia Epithelial Cells. Kidney. Polycystic Kidney, Autosomal Recessive Sodium Sodium Channels'
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Journal articles on the topic "Cilia Epithelial Cells. Kidney. Polycystic Kidney, Autosomal Recessive Sodium Sodium Channels"
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Kawa, G., S. Nagao, A. Yamamoto, K. Omori, Y. Komatz, H. Takahashi, and Y. Tashiro. "Sodium pump distribution is not reversed in the DBA/2FG-pcy, polycystic kidney disease model mouse." Journal of the American Society of Nephrology 4, no. 12 (June 1994): 2040–49. http://dx.doi.org/10.1681/asn.v4122040.
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Recently, it has been reported that Na,K-ATPase in the renal epithelia of human autosomal dominant polycystic kidney disease and cpk mouse, a murine model of autosomal recessive polycystic kidney disease, mislocates to apical plasma membrane and that mislocated Na,K-ATPase causes the cyst formation. Whether the DBA/2FG-pcy mice, which are presumably a suitable model for autosomal dominant polycystic kidney disease, also exhibit the reversal polarity of Na,K-ATPase localization was examined. Kidneys of newborn DBA/2FG-pcy mice, and those at early and late stages of cyst development were examined by immunohistochemical techniques. At any stage, abnormal distribution of Na,K-ATPase on the apical membranes of tubular epithelial cells could not be detected. It is suggested that cysts can be formed without reversed polarity of Na,K-ATPase distribution in pcy mice.
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Rohatgi, Rajeev, Lorenzo Battini, Paul Kim, Sharon Israeli, Patricia D. Wilson, G. Luca Gusella, and Lisa M. Satlin. "Mechanoregulation of intracellular Ca2+ in human autosomal recessive polycystic kidney disease cyst-lining renal epithelial cells." American Journal of Physiology-Renal Physiology 294, no. 4 (April 2008): F890—F899. http://dx.doi.org/10.1152/ajprenal.00341.2007.
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Mutations of cilia-expressed proteins are associated with an attenuated shear-induced increase in intracellular Ca2+ concentration ([Ca2+]i) in renal epithelial cell lines derived from murine models of autosomal recessive polycystic kidney disease (ARPKD). We hypothesized that human ARPKD cyst-lining renal epithelial cells also exhibited dysregulated mechanosensation. To test this, conditionally immortalized cell lines derived from human fetal ARPKD cyst-lining (pool and clone 5E) cell lines with low levels of fibrocystin/polyductin expression and age-matched normal collecting tubule [human fetal collecting tubule (HFCT) pool and clone 2C] cell lines were grown in culture, loaded with a Ca2+ indicator dye, and subjected to laminar shear. Clonal cell lines were derived from single cells present in pools of cells from cyst-lining and collecting tubules, microdissected from human kidney. Resting and peak [Ca2+]i were similar between ARPKD 5E and pool, and HFCT 2C and pool; however, the flow-induced peak [Ca2+]i was greater in ARPKD 5E (700 ± 87 nM, n = 21) than in HFCT 2C (315 ± 58 nM, n = 12; P < 0.01) cells. ARPKD 5E cells treated with Gd3+, an inhibitor of nonselective cation channels, inhibited but did not abolish the shear-induced [Ca2+]i transient. Cilia were ∼20% shorter in ARPKD than HFCT cells, but no difference in ciliary localization or total cellular expression of polycystin-2, a mechanosenory Gd3+-sensitive cation channel, was detected between ARPKD and HFCT cells. The intracellular Ca2+ stores were similar between cells. In summary, human ARPKD cells exhibit an exaggerated Gd3+-sensitive mechano-induced Ca2+ response compared with controls; whether this represents dysregulated polycystin-2 activity in ARPKD cells remains to be explored.
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Liu, Wen, Noel S. Murcia, Yi Duan, Sheldon Weinbaum, Bradley K. Yoder, Erik Schwiebert, and Lisa M. Satlin. "Mechanoregulation of intracellular Ca2+ concentration is attenuated in collecting duct of monocilium-impaired orpk mice." American Journal of Physiology-Renal Physiology 289, no. 5 (November 2005): F978—F988. http://dx.doi.org/10.1152/ajprenal.00260.2004.
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Autosomal recessive polycystic kidney disease (ARPKD) is characterized by the progressive dilatation of collecting ducts, the nephron segments responsible for the final renal regulation of sodium, potassium, acid-base, and water balance. Murine models of ARPKD possess mutations in genes encoding cilia-associated proteins, including Tg737 in orpk mice. New findings implicate defects in structure/function of primary cilia as central to the development of polycystic kidney disease. Our group (Liu W, Xu S, Woda C, Kim P, Weinbaum S, and Satlin LM, Am J Physiol Renal Physiol 285: F998–F1012, 2003) recently reported that increases in luminal flow rate in rabbit collecting ducts increase intracellular Ca2+ concentration ([Ca2+]i) in cells therein. We thus hypothesized that fluid shear acting on the apical membrane or hydrodynamic bending moments acting on the cilium increase renal epithelial [Ca2+]i. To further explore this, we tested whether flow-induced [Ca2+]i transients in collecting ducts from mutant orpk mice, which possess structurally abnormal cilia, differ from those in controls. Isolated segments from 1- and 2-wk-old mice were microperfused in vitro and loaded with fura 2; [Ca2+]i was measured by digital ratio fluorometry before and after the rate of luminal flow was increased. All collecting ducts responded to an increase in flow with an increase in [Ca2+]i, a response that appeared to be dependent on luminal Ca2+ entry. However, the magnitude of the increase in [Ca2+]i in 2- but not 1-wk-old mutant orpk animals was blunted. We speculate that this defect in mechano-induced Ca2+ signaling in orpk mice leads to aberrant structure and function of the collecting duct in ARPKD.
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Veizis, Elias I., Cathleen R. Carlin, and Calvin U. Cotton. "Decreased amiloride-sensitive Na+ absorption in collecting duct principal cells isolated from BPK ARPKD mice." American Journal of Physiology-Renal Physiology 286, no. 2 (February 2004): F244—F254. http://dx.doi.org/10.1152/ajprenal.00169.2003.
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The main feature of polycystic kidney diseases (PKD) is formation and progressive enlargement of renal cysts. Alterations in epithelial cell proliferation, extracellular matrix, and ion transport are thought to contribute to cyst enlargement and loss of renal function. Abnormal Cl- secretion is implicated in cyst enlargement in autosomal dominant PKD (ADPKD), but little is known about transport abnormalities in autosomal recessive PKD (ARPKD). We developed a method to isolate collecting duct (CD) principal cells (site of the lesion in ARPKD) from normal and ARPKD mice. A transgenic mouse (Hoxb7/GFP) in which enhanced green fluorescent protein (GFP) is expressed in CDs was bred with an ARPKD mouse (BPK), and GFP-positive cells from normal and cystic mice were selected by fluorescence-activated cell sorting. GFP-positive CD cells (>95 ± 3%) obtained from either normal or cystic mice formed high-resistance, polarized epithelial monolayers. Expression patterns for marker proteins and the presence of a central cilium confirmed that the monolayers are composed of principal cells. Under basal conditions, the Cl- secretory responses elicited by elevation of cAMP or calcium were not significantly different between normal and cystic monolayers. In contrast, the amiloride-sensitive short-circuit current was significantly reduced in monolayers of cells isolated from cystic mice (12.9 ± 1.6 μA/cm2; n = 10) compared with monolayers of cells isolated from normal mice (27.3 ± 3.4 μA/cm2; n = 12). The results of these studies suggest that epithelial sodium channel-mediated sodium absorption is decreased in principal cells of ARPKD CD cysts and that the reduction in sodium absorption may contribute to the accumulation of luminal fluid.
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Veizis, I. Elias, and Calvin U. Cotton. "Abnormal EGF-dependent regulation of sodium absorption in ARPKD collecting duct cells." American Journal of Physiology-Renal Physiology 288, no. 3 (March 2005): F474—F482. http://dx.doi.org/10.1152/ajprenal.00227.2004.
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Amiloride-sensitive sodium entry, via the epithelial sodium channel (ENaC), is the rate-limiting step for Na+ absorption in kidney collecting ducts, and epidermal growth factor (EGF) inhibits Na+ transport and ENaC expression. A pathognomonic feature of polycystic kidney disease (PKD) is EGF receptor mislocalization to the apical plasma membrane and EGF/EGF receptor axis overactivity. Immunohistochemical and biochemical analysis revealed mislocalization of EGF receptor and excessive activation of the p42/44 extracellular signal-regulated protein kinase pathway (ERK1/2) in kidneys from cystic mice compared with noncystic littermates. Primary monolayer cultures of noncystic and cystic murine collecting duct principal cells were used to identify aberrant EGF-dependent ERK1/2 activation and regulation of Na+ transport associated with autosomal recessive PKD. Addition of EGF to the basolateral bathing solution of noncystic or cystic monolayers led to p42/44 phosphorylation and inhibition of Na+ transport (30–35%), whereas apical EGF was effective only in monolayers derived from cystic mice. p42/44 Phosphorylation and inhibition of Na+ transport were prevented by prior treatment of the cells with an ERK kinase inhibitor. Chronic treatment (24 h) of noncystic and cystic monolayers with basolateral EGF elicited sustained inhibition of Na+ absorption (50–55%) and a reduction in steady-state ENaC mRNA levels (50–75%). In contrast, addition of EGF to the apical bathing solution (24 h) had no effect in noncystic monolayers but led to inhibition of Na+ transport (50–60%) and decreased ENaC expression (45–60%) in cystic cells. Pretreatment of the monolayers with an ERK kinase inhibitor abolished the chronic effects of EGF on Na+ transport. The results of these studies reveal that the mislocalized apical EGF receptors are functionally coupled to the ERK pathway and that abnormal EGF-dependent regulation of ENaC function and expression may contribute to PKD pathophysiology.
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Sweeney, William E., Linda Kusner, Cathleen R. Carlin, Sharon Chang, Lidia Futey, Calvin U. Cotton, Katherine MacRae Dell, and Ellis D. Avner. "Phenotypic analysis of conditionally immortalized cells isolated from the BPK model of ARPKD." American Journal of Physiology-Cell Physiology 281, no. 5 (November 1, 2001): C1695—C1705. http://dx.doi.org/10.1152/ajpcell.2001.281.5.c1695.
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To study the pathophysiology of autosomal recessive polycystic kidney disease (ARPKD), we sought to develop conditionally immortalized control and cystic murine collecting tubule (CT) cell lines. CT cells were isolated from intercross breedings between BPK mice ( bpk +/−), a murine model of ARPKD, and the Immorto mice (H-2Kb- ts-A58 +/+ ). Second-generation outbred offspring (BPK × Immorto) homozygous for the BPK mutation ( bpk −/−; Im +/±; cystic BPK/H-2Kb- ts-A58), were phenotypically indistinguishable from inbred cystic BPK animals ( bpk −/−). Cystic BPK/H-2Kb- ts-A58 mice developed biliary ductal ectasia and massively enlarged kidneys, leading to renal failure and death by postnatal day 24. Principal cells (PC) were isolated from outbred cystic and noncystic BPK/H-2Kb- ts-A58 littermates at specific developmental stages. Epithelial monolayers were under nonpermissive conditions for markers of epithelial cell polarity and PC function. Cystic and noncystic cells displayed several properties characteristic of PCs in vivo, including amiloride-sensitive sodium transport and aquaporin 2 expression. Cystic cells exhibited apical epidermal growth factor receptor (EGFR) mislocalization but normal expression of ZO-1 and E-cadherin. Hence, these cell lines retain the requisite characteristics of PCs, and cystic BPK/H-2Kb- ts-A58 PCs retained the abnormal EGFR membrane expression characteristic of ARPKD. These cell lines represent important new reagents for studying the pathogenesis of ARPKD.
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Wilson, P. D. "Epithelial cell polarity and disease." American Journal of Physiology-Renal Physiology 272, no. 4 (April 1, 1997): F434—F442. http://dx.doi.org/10.1152/ajprenal.1997.272.4.f434.
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The establishment and maintenance of epithelial polarity is essential for the integrity and function of epithelial organs and is particularly critical in the kidney, where vectorial reabsorption and secretion are effected in different segments of the nephron by the differential polarized insertion of channels, transporters, and related proteins into apical membranes lining the tubule lumen or basolateral membranes adjacent to the interstitium and blood space. Faulty intracellular delivery and polarization of membrane proteins can lead to serious diseases such as cystic fibrosis, I cell disease, and renal cystic diseases. The best understood disease of epithelial polarity is autosomal dominant polycystic kidney disease (ADPKD) caused by mutations in a >462-kDa, developmentally regulated membrane protein, “polycystin.” ADPKD cysts are characteristically lined by a single layer of structurally polarized epithelial cells with normal functional intercellular tight junctions but with aberrant polarization of some important membrane proteins. Abnormal apical membrane polarity of biochemically active, ouabain-sensitive Na-K-adenosinetriphosphatase (Na-K-ATPase) in ADPKD cyst epithelia leads to abnormal sodium ion secretion and provides a mechanism for aberrant fluid secretion. In addition, apically mislocated, functional epidermal growth factor (EGF) receptors on cyst epithelia, together with EGF synthesis and secretion into cyst lumens, provide a mechanism for autocrine regulation of increased epithelial cell proliferation in ADPKD. Underlying mechanisms for these abnormalities in polarized distribution of membrane proteins include the aberrant expression of fetal gene products, such as the beta2-subunit of Na-K-ATPase, in ADPKD kidneys. Overexpression of polycystin protein in ADPKD cyst epithelia, low levels restricted to medullary collecting tubules in normal adult kidneys, and high levels in ureteric bud-derived structures in human fetal kidneys further suggest a failure of downregulation of fetal genes as a mechanism for the polarity abnormalities that characterize ADPKD.
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Li, Fang, Xiao-Qing Dai, Qiang Li, Yuliang Wu, and Xing-Zhen Chen. "Inhibition of polycystin-L channel by the Chinese herb Sparganum stoloniferum Buch.-Ham." Canadian Journal of Physiology and Pharmacology 84, no. 8-9 (September 2006): 923–27. http://dx.doi.org/10.1139/y06-040.
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The Chinese herb Sparganum stoloniferum Buch.-Ham. (SBH) is frequently used to improve blood circulation and to rehabilitate vascular obstruction in traditional Chinese medicine. It was recently reported that SBH reduces the proliferation of renal epithelial cells stimulated by epidermal growth factor (EGF), and inhibits the phosphorylation of the EGF receptor. SBH has also been used as a trial drug to treat polycystic kidney disease (PKD) patients in China. The potential molecular actions of SBH on PKD remain unknown. Autosomal dominant PKD (ADPKD) is associated with mutations in polycystin-1 or polycystin-2 (PC2). PC2 and its homologue, polycystin-L (PCL), are nonselective cation channels permeable to potassium, sodium, and calcium. Here, we examine the effects of SBH on the human PCL channel expressed in Xenopus oocytes, using 2-microelectrode voltage-clamp electrophysiology and radiotracer uptake measurements. In PCL-expressing oocytes, with or without preincubation with SBH, the PCL channel was inhibited by SBH in a dose-dependent and reversible manner; a concentration of 2% SBH completely abolished the channel activation. The IC50 value for SBH was 0.48% ± 0.03%, with a 10-min preincubation period. SBH was also found to inhibit the PCL-mediated 45Ca tracer uptake in oocytes. Our study suggests that SBH contains 1 or more yet-to-be determined components that are inhibitors of PCL channel. The therapeutic potential of SBH for ADPKD and its chemical composition remain to be investigated.
Dissertations / Theses on the topic "Cilia Epithelial Cells. Kidney. Polycystic Kidney, Autosomal Recessive Sodium Sodium Channels"
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Olteanu, Dragos S. "Dysregulated ENAC and NHE function in cilium-deficient renal collecting duct cell monolayers a model of polycystic kidney disease /." Thesis, Birmingham, Ala. : University of Alabama at Birmingham, 2007. https://www.mhsl.uab.edu/dt/2009r/olteanu.pdf.
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