Abstract:
:Gain-of-function variants in CFB encoding factor B (FB), a component of the alternative pathway C3 convertase, have been reported in a minority of patients with aHUS and result in massive C3 activation. Zhang et al. describe the functional characterization of a novel FB variant (p.Ser367Arg) that they identified in 2 unrelated aHUS pedigrees who had undetectable C3 levels. The mutant FB caused strong C3 cleavage in fluid-phase but also C3 deposition on cell surface. This commentary addresses the implications of these findings for understanding the complexity of complement-related genetic renal diseases.
journal_name
Kidney Intjournal_title
Kidney internationalauthors
Noris M,Remuzzi Gdoi
10.1016/j.kint.2020.06.026subject
Has Abstractpub_date
2020-11-01 00:00:00pages
1084-1087issue
5eissn
0085-2538issn
1523-1755pii
S0085-2538(20)30814-0journal_volume
98pub_type
评论,杂志文章abstract::Vascular sclerosis is often seen in renal biopsies. It is usually associated with diabetes mellitus, hypertension, smoking, etc. However, whether inherited thrombophilic states such as factor V gene mutation, prothrombin gene mutation, and methylenetetrahydrofolate reductase (MTHFR) gene mutation are associated with t...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/sj.ki.5001551
更新日期:2006-08-01 00:00:00
abstract::The present study examined the effect of elevated glucose on glomerular cell membrane lipid peroxidation. Glomeruli were isolated from male Sprague-Dawley rat kidneys utilizing a standard sieving method and incubated in medium containing different concentrations of glucose at 37 degrees C for one and 48 hours. The lev...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1994.461
更新日期:1994-12-01 00:00:00
abstract:BACKGROUND:The ontogeny of the renal mineralocorticoid (MR) and glucocorticoid (GR) receptors in the ovine fetus, and the effects of early exposure to synthetic or natural glucocorticoids on the expression of these genes in late gestation were examined. METHODS:A partial cDNA sequence for the ovine MR was cloned and u...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1046/j.1523-1755.2002.00157.x
更新日期:2002-02-01 00:00:00
abstract::The aging process in humans and in the rat is associated with an impairment in renal tubular reabsorption of Pi and renal tubular adaptation to a low Pi diet. The purposes of the present study were to determine whether changes in the abundance of type II Na-Pi contransporter (NaPi-2) protein and/or mRNA play a role in...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1996.385
更新日期:1996-09-01 00:00:00
abstract::Dysregulated production of adipocytokines may be involved in the development of atherosclerotic cardiovascular disease in metabolic syndrome and chronic kidney disease (CKD) associated with metabolic syndrome. The aim of this study was to determine the effects of treatment with angiotensin II (Ang II) type-1 receptor ...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/sj.ki.5001810
更新日期:2006-11-01 00:00:00
abstract::With a lifetime incidence of up to 12% in man and 6% in woman, nephrolithiasis is a major health problem worldwide. Approximately, 80% of kidney stones are composed of calcium and hypercalciuria is found in up to 40% of stone-formers. Although the mechanisms resulting in precipitation and growth of calcium crystals in...
journal_title:Kidney international
pub_type: 杂志文章,评审
doi:10.1038/sj.ki.5002441
更新日期:2007-11-01 00:00:00
abstract::Intestinal regulation of oxalate absorption is a complex mechanism, not exclusively reliant on the oxalate-degrading anaerobe Oxalobacter formigenes. Using metagenomics, Miller et al. were able to describe a network of bacterial taxa co-occurring with Oxalobacter formigenes in fecal samples from non-stone forming cont...
journal_title:Kidney international
pub_type: 评论,杂志文章
doi:10.1016/j.kint.2019.03.020
更新日期:2019-07-01 00:00:00
abstract::The role of glutathione (GSH) in the preservation of renal function and the pathogenesis of renal injury has been investigated using the isolated perfused rat kidney as a model. In kidneys perfused for 80 min with 5 mM glucose as the only exogenous substrate, tissue GSH becomes depleted, renal function deteriorates, a...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1983.142
更新日期:1983-08-01 00:00:00
abstract::To determine whether the induction of immune-mediated glomerular injury influences the formation of cyclooxygenase products by glomerular cells, we determined prostaglandin E2 (PGE2) and thromboxane B2 (TXB2) (as the stable metabolite of TXA2) formation in isolated glomeruli of rats with passive Heymann nephritis (PHN...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1987.118
更新日期:1987-05-01 00:00:00
abstract::Mice chronically infected with the virus of lymphocytic choriomeningitis (LCM) develop immune complex glomerulonephritis. Others have shown that adoptive immunization of these mice by the i.p. injection of syngeneic immune spleen cells terminates the chronic viral carrier state. The present studies were designed to de...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1977.49
更新日期:1977-05-01 00:00:00
abstract::Mutations in the complement regulatory proteins are associated with several different diseases. Although these mutations cause dysregulated alternative pathway activation throughout the body, the kidneys are the most common site of injury. The susceptibility of the kidney to alternative pathway-mediated injury may be ...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1016/j.kint.2016.02.036
更新日期:2016-07-01 00:00:00
abstract::When patients develop acute kidney injury, a small fraction of them will develop end-stage renal disease later. The severity of renal impairment in the remaining patients is uncertain because studies have not carefully examined renal function over time or the precise timing of entry into a late stage of chronic kidney...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.2009.332
更新日期:2009-11-01 00:00:00
abstract::The impact of dialysis on cognitive trends is uncertain. Kurella Tamura et al. evaluated cognitive trajectories in 212 participants from the Chronic Renal Insufficiency Cohort study and found that dialysis initiation was associated with a decline in executive function. This is consistent with a cerebrovascular basis f...
journal_title:Kidney international
pub_type: 评论,杂志文章
doi:10.1016/j.kint.2017.01.022
更新日期:2017-04-01 00:00:00
abstract::Pain is a common complaint in patients with autosomal-dominant polycystic kidney disease, and a systematic approach is needed to differentiate the etiology of the pain and define an approach to management. A thorough history is the best clue to the multifactorial causes of the pain, superimposed upon an understanding ...
journal_title:Kidney international
pub_type: 杂志文章,评审
doi:10.1046/j.1523-1755.2001.00985.x
更新日期:2001-11-01 00:00:00
abstract::Although case reports link proton-pump inhibitor (PPI) use and hypomagnesemia, no large-scale studies have been conducted. Here we examined the serum magnesium concentration and the likelihood of hypomagnesemia (<1.6 mg/dl) with a history of PPI or histamine-2 receptor antagonist used to reduce gastric acid, or use of...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.2012.452
更新日期:2013-04-01 00:00:00
abstract::Fibroblast growth factor-23 (FGF23), a regulator of mineral metabolism, has been linked to cardiovascular disease in chronic kidney disease. As community-based data of the longitudinal association between FGF23 and cardiovascular events are lacking, we investigated a possible relationship in 727 men of the Uppsala Lon...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.2012.327
更新日期:2013-01-01 00:00:00
abstract::In uremic patients resistance to the action of insulin has been documented, but it is not known at what stage of renal disease it appears. We therefore examined 29 patients with IgA glomerulonephritis (IgAGN) and 21 patients with adult polycystic kidney disease (ADPKD) in different stages of renal failure, and in addi...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1046/j.1523-1755.1998.00898.x
更新日期:1998-05-01 00:00:00
abstract:INTRODUCTION:Thiazide diuretics have the unique characteristic of increasing renal Na+ excretion, while decreasing Ca2+ excretion. However, the molecular mechanism responsible for this thiazide-induced hypocalciuria remains unclear. The present study investigates the effect of thiazides on the expression of the protein...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1046/j.1523-1755.2003.00128.x
更新日期:2003-08-01 00:00:00
abstract::Progressive kidney fibrosis precedes end-stage renal failure in up to a third of patients with diabetes mellitus. Elevated intra-renal transforming growth factor-beta (TGF-beta) is thought to underlie disease progression by promoting deposition of extracellular matrix and epithelial-mesenchymal transition. GW788388 is...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/sj.ki.5002717
更新日期:2008-03-01 00:00:00
abstract::Lysophosphatidic acid (LPA) is known to regulate various biological responses by binding to LPA receptors. The serum level of LPA is elevated in diabetes, but the involvement of LPA in the development of diabetes and its complications remains unknown. Therefore, we studied LPA signaling in diabetic nephropathy and the...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1016/j.kint.2016.11.010
更新日期:2017-06-01 00:00:00
abstract::The following bone density measurements have limited utility in determining bone strength because they do not include bone quality: microarchitecture, mineralization, ability to repair damage, collagen structure, crystal size, or marrow composition. Patients with kidney disease have poor bone quality. Newman et al. no...
journal_title:Kidney international
pub_type: 评论,杂志文章
doi:10.1016/j.kint.2015.11.004
更新日期:2016-01-01 00:00:00
abstract::Renin secretion was compared in vivo and in vitro among five groups of rats, each group subjected to a different sodium balance for 10 to 14 days. The state of the renin-angiotensin system in vivo was evaluated by measuring the renal renin (RR) and the plasma renin concentration (PRC) in both anesthetized and nonanest...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1980.71
更新日期:1980-05-01 00:00:00
abstract::In a rat model of glomerular immune injury induced by administration of anti-glomerular basement membrane antibody and resembling human rapidly progressive glomerulonephritis, we explored whether activation of inducible nitric oxide synthase (iNOS) regulates synthesis of eicosanoids originating from cyclooxygenation o...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1046/j.1523-1755.1998.00791.x
更新日期:1998-03-01 00:00:00
abstract::How the dialysis dose is normalized is just one of several assumptions that clinicians need to take into account when prescribing peritoneal dialysis. El-Kateb et al. confirm that estimating the volume of urea distribution is associated with significant error and show that energy expenditure is not linearly related to...
journal_title:Kidney international
pub_type: 评论,杂志文章
doi:10.1016/j.kint.2016.09.020
更新日期:2016-12-01 00:00:00
abstract::Because of high risk of cardiovascular disease, patients with chronic kidney disease may benefit from cholesterol-lowering therapy beyond statins. A cost-effectiveness analysis of adding ezetimibe to high-dose statins for primary cardiovascular disease prevention in patients with non-dialysis-dependent chronic kidney ...
journal_title:Kidney international
pub_type: 评论,杂志文章
doi:10.1016/j.kint.2019.02.029
更新日期:2019-07-01 00:00:00
abstract::Leucocyte interaction with other cells is facilitated by the adhesion molecules, leucocyte function-associated antigen-1 (LFA-1)-binding to intercellular adhesion molecule-1 (ICAM-1) and for T cells and natural killer (NK) cells the binding of LFA-2 (CD2) to LFA-3. As these interactions are critical for the mediation ...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/ki.1989.303
更新日期:1989-12-01 00:00:00
abstract::We have shown that cisplatin inhibits fatty acid oxidation, and that fibrate treatment ameliorates renal function by preventing the inhibition of fatty acid oxidation and proximal tubule cell death. Urine samples of mice treated with single injection of cisplatin (20 mg/kg body weight) were collected for 3 days and an...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1038/sj.ki.5000433
更新日期:2006-06-01 00:00:00
abstract::Early identification of kidney transplant recipients at risk of progressive allograft dysfunction may allow clinicians to provide closer monitoring and more aggressive risk factor modification. In this issue, Raynaud et al. presented a latent class model that clustered kidney transplant recipients into 8 risk categori...
journal_title:Kidney international
pub_type: 评论,杂志文章
doi:10.1016/j.kint.2020.07.031
更新日期:2021-01-01 00:00:00
abstract::Propofol has been shown to provide protection against renal ischemia/reperfusion injury experimentally, but clinical evidence is lacking. Here we studied the effect of propofol anesthesia on the occurrence of acute kidney injury following heart surgery with cardiopulmonary bypass. One hundred and twelve patients who u...
journal_title:Kidney international
pub_type: 杂志文章,随机对照试验
doi:10.1038/ki.2013.532
更新日期:2014-08-01 00:00:00
abstract:BACKGROUND:Angiotensin II (Ang II) can up-regulate nicotinamide adenine dinucleotide phosphate [NAD(P)H] oxidase, whose product superoxide anion (O2-) can interact with nitric oxide (NO) to form peroxynitrite (ONOO-). We tested the hypothesis that Ang II subtype 1 (AT1) receptor activation enhances oxidative stress and...
journal_title:Kidney international
pub_type: 杂志文章
doi:10.1046/j.1523-1755.2002.00123.x
更新日期:2002-01-01 00:00:00