Abstract：
BACKGROUND:Uromodulin (Tamm-Horsfall protein) is the most abundant urinary protein in healthy individuals. Despite 60 years of research, its physiological role remains rather elusive. Familial juvenile hyperuricemic nephropathy and medullary cystic kidney disease Type 2 are autosomal dominant tubulointerstitial nephropathies characterized by gouty arthritis and progressive renal insufficiency, caused by uromodulin (UMOD) mutations. The aim of this study was to compare the cellular effects of mutant and wild-type UMOD. METHODS:Wild-type UMOD cDNA was cloned from human kidney cDNA into pcDNA3 expression vector. A mutant UMOD construct, containing the previously reported mutation, V273, was created by in vitro mutagenesis. Transient and stable transfection studies were performed in human embryonic kidney cells and mouse distal convoluted tubular cells, respectively. Expression was evaluated by reverse transcription polymerase chain reaction (RT-PCR), western blot and immunofluorescence. Oligosaccharide cleavage by glycosidases was performed to characterize different forms of UMOD. Nuclear translocation of P65 and degradation of IκBα and IRAK1 in response to interleukin (IL)-1β were used to evaluate the effects of wild-type and mutant UMOD on the IL-1R-NFκB pathway. RESULTS:The mutant protein was shown to be retained in the endoplasmic reticulum and was not excreted to the cell medium, as opposed to the wild-type protein. NFκB activation in cells expressing mutant UMOD was similar to that of untransfected cells. In contrast, cells over-expressing wild-type UMOD showed markedly reduced NFκB activation. CONCLUSION:Our findings suggest that UMOD may have a physiologic function related to its inhibitory effect on the NFκB pathway.
journal_name
J Nephroljournal_title
Journal of nephrologyauthors
Dinour D,Ganon L,Nomy LI,Ron R,Holtzman EJdoi
10.1007/s40620-014-0079-7subject
Has Abstractpub_date
2014-06-01 00:00:00pages
257-64issue
3eissn
1121-8428issn
1724-6059journal_volume
27pub_type
杂志文章abstract：BACKGROUND:Incremental dialysis may preserve residual renal function and improve survival in comparison with full-dose dialysis; however, available evidence is limited. We therefore compared all-cause mortality and residual kidney function (RKF) loss in incremental and full-dose dialysis and time to full-dose dialysis ...
journal_title：Journal of nephrology
pub_type： 杂志文章,meta分析
doi：10.1007/s40620-018-00577-9
更新日期：2019-10-01 00:00:00
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journal_title：Journal of nephrology
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doi：10.1007/s40620-016-0324-3
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journal_title：Journal of nephrology
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journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-015-0212-2
更新日期：2016-02-01 00:00:00
abstract：:Page kidney was first described in animal experiments in 1939. In the 1950s and 1960s the human counterpart became evident. In this review we examine the modest literature on this rare but important renal/urological complication, summarize the clinical features, and discuss the best approach to diagnosis and managemen...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：2003-05-01 00:00:00
abstract：:Lupus is now regarded as a syndrome which results from several related auto-immune processes, although the exact mechanisms of how the disease arises in susceptible individuals remain obscure. When the San Carlo meetings began in 1968, much less was known about the pathogenesis of autoimmunity, but the presence of aut...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：1999-07-01 00:00:00
abstract：BACKGROUND:Acute kidney injury (AKI) following acute ST elevation myocardial infarction (STEMI) is associated with adverse outcomes. The recently proposed KDIGO criteria suggested modifications to the consensus classification system for AKI, namely lowering the threshold of increase in absolute serum creatinine and ext...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-017-0461-3
更新日期：2018-06-01 00:00:00
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journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2003-11-01 00:00:00
abstract：:One of the major concerns in organ transplantation is the early detection of humoral rejection, through improved diagnostic and prognostic biomarkers. Long-term survival of renal allografts is significantly lower in recipients developing donor-specific anti-HLA antibodies (DSAs) either pretransplant or posttransplant....
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：10.5301/jn.5000240
更新日期：2013-03-01 00:00:00
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journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2008-03-01 00:00:00
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journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2009-07-01 00:00:00
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journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：1997-01-01 00:00:00
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journal_title：Journal of nephrology
pub_type： 临床试验,杂志文章
doi：
更新日期：2007-07-01 00:00:00
abstract：BACKGROUND:Cardiovascular (CV) diseases are the main cause of death in maintenance hemodialysis (MHD) patients. Muscle wasting and physical function decline are common in MHD patients, and significantly impair their quality of life. These can result from abnormalities in cardiac function, which can be further worsened ...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-015-0188-y
更新日期：2015-12-01 00:00:00
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journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2004-09-01 00:00:00
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journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：2009-11-01 00:00:00
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journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：10.5301/jn.5000025
更新日期：2011-11-01 00:00:00
abstract：:In this paper we concentrate on the role of infections in IgA nephropathy both from a pathogenetic and clinic point of view. The current hypotheses as regards the role of infections in the pathogenesis of IgA nephropathy are: (a) role of particular pathogens, (b) chronic exposure to mucosal infections, (c) abnormal ha...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：10.1007/s40620-016-0265-x
更新日期：2016-08-01 00:00:00
abstract：BACKGROUND:The effect of uric acid on nocturnal dipping in hypertensive patients is unknown. We analyzed the specific relationship between uric acid and nocturnal dipping status in newly diagnosed essential hypertensive patients with normal renal function. METHODS:Two hundred fifteen patients with newly diagnosed esse...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2008-05-01 00:00:00
abstract：BACKGROUND AND METHODS:Smoking is associated with hyperparathyroidism in the elderly general population and nicotine, the main component of tobacco smoke, stimulates PTH release in experimental models. Although smoking is a persisting problem in patients with end-stage renal disease (ESRD), the association between smok...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.5301/JN.2011.7740
更新日期：2012-01-01 00:00:00
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pub_type： 杂志文章
doi：
更新日期：2007-09-01 00:00:00
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pub_type： 杂志文章,评审
doi：10.5301/jn.5000233
更新日期：2013-07-01 00:00:00
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doi：
更新日期：2010-09-01 00:00:00
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journal_title：Journal of nephrology
pub_type： 杂志文章,meta分析,评审
doi：10.1007/s40620-014-0085-9
更新日期：2014-08-01 00:00:00
abstract：BACKGROUND:The detailed mechanisms and treatment methods of chronic kidney disease (CKD) in patients with chronic heart failure (CHF) are not fully understood. We conducted a prospective study in CHF patients manifesting CKD to examine if AST-120 (Kremezin) improves cardiac and renal functions. METHODS:Twenty outpatie...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2010-09-01 00:00:00
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journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：2006-03-01 00:00:00
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journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2006-05-01 00:00:00
abstract：OBJECTIVE:Chronic kidney disease (CKD) pregnancies are at high risk of developing adverse outcomes. In non-pregnant subjects with CKD, higher urinary IgM levels are associated with poor renal survival and higher rates of cardiovascular deaths. In this study, we assessed whether urinary IgM levels are associated with an...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-018-0536-9
更新日期：2019-04-01 00:00:00
abstract：INTRODUCTION:Several studies have suggested an effect of calcineurin inhibitors on arterial compliance in kidney transplant recipients. We aimed to investigate whether the type of calcineurin inhibitor influences large and small artery compliance in these patients independently of other determinants. METHODS:Patients ...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2008-01-01 00:00:00
abstract：BACKGROUND:Dialysis-requiring acute kidney injury (D-AKI) is common in hospitalized patients. Many patients survive the immediate post AKI period, thus at risk of suffering long-term sequelae of AKI. Prior studies examining long term outcomes lack non-dialyzed AKI control groups. Without non-dialyzed AKI control group,...
journal_title：Journal of nephrology
pub_type： 杂志文章
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更新日期：2016-12-01 00:00:00
