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Glomerular basement membrane

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https://www.readbyqxmd.com/read/28814668/injury-induced-actin-cytoskeleton-reorganization-in-podocytes-revealed-by-super-resolution-microscopy
#1
Hani Y Suleiman, Robyn Roth, Sanjay Jain, John E Heuser, Andrey S Shaw, Jeffrey H Miner
The architectural integrity of tissues requires complex interactions, both between cells and between cells and the extracellular matrix. Fundamental to cell and tissue homeostasis are the specific mechanical forces conveyed by the actomyosin cytoskeleton. Here we used super-resolution imaging methods to visualize the actin cytoskeleton in the kidney glomerulus, an organized collection of capillaries that filters the blood to make the primary urine. Our analysis of both mouse and human glomeruli reveals a network of myosin IIA-containing contractile actin cables within podocyte cell bodies and major processes at the outer aspects of the glomerular tuft...
August 17, 2017: JCI Insight
https://www.readbyqxmd.com/read/28813476/paratubular-basement-membrane-insudative-lesions-predict-renal-prognosis-in-patients-with-type-2-diabetes-and-biopsy-proven-diabetic-nephropathy
#2
Koki Mise, Yutaka Yamaguchi, Junichi Hoshino, Toshiharu Ueno, Akinari Sekine, Keiichi Sumida, Masayuki Yamanouchi, Noriko Hayami, Tatsuya Suwabe, Rikako Hiramatsu, Eiko Hasegawa, Naoki Sawa, Takeshi Fujii, Shigeko Hara, Hitoshi Sugiyama, Hirofumi Makino, Jun Wada, Kenichi Ohashi, Kenmei Takaichi, Yoshifumi Ubara
AIMS: Glomerular insudative lesions are a pathological hallmark of diabetic nephropathy (DN). However, paratubular basement membrane insudative lesions (PTBMIL) have not attracted much attention, and the association between such lesions and the renal prognosis remains unclear. METHODS: Among 142 patients with biopsy-proven DN and type 2 diabetes encountered from 1998 to 2011, 136 patients were enrolled in this study. Patients were classified into 3 groups (Group 1: mild, Group 2: moderate, Group 3: severe) according to the extent of cortical and medullary PTBMIL...
2017: PloS One
https://www.readbyqxmd.com/read/28807257/revisiting-the-determinants-of-the-glomerular-filtration-barrier-what-goes-round-must-come-round
#3
Detlef Schlöndorff, Christina M Wyatt, Kirk N Campbell
The glomerular filtration barrier (GFB) is characterized by a very high hydraulic permeability, combined with a marked permselectivity that excludes macromolecules such as albumin. Thus, the GFB retains most of the plasma proteins, with only 0.06% of albumin getting across the basement membrane. The GFB consists of 3 layers: fenestrated endothelial cells, the glomerular basement membrane, and podocytes. Injury to any of these components can result in the development of proteinuria. The contribution of the major components of the GFB has recently been reexamined and is discussed in the context of our past and present understanding...
September 2017: Kidney International
https://www.readbyqxmd.com/read/28796565/ultrastructural-changes-of-kidney-in-schistosoma-mansoni-infected-mice
#4
Refaat A Eid, Mubarak Al-Shraim, Fahmy El-Sayed, Khaled Radad
Schistosomiasis is the second threatening parasitic disease after malaria and among Schistosoma spp., Schistosoma mansoni (S. mansoni) affects about 100 million people in tropic regions in Africa and South America. The current study was carried out to investigate ultrastructural changes of the kidney in mice infected with cercariae of S. mansoni, in which 20 Swiss albino mice of 60-day-old were assigned into two groups (10 each). Control group received 1 ml normal saline by intraperitoneal route. Model group were intraperitoneally infected with 1 ml normal saline containing 40 cercariae of S...
August 10, 2017: Ultrastructural Pathology
https://www.readbyqxmd.com/read/28796467/anti-glomerular-basement-membrane-antibody-diagnostics-in-a-large-cohort-tertiary-center-should-we-trust-serological-findings
#5
Abdulla Watad, Nicola Luigi Bragazzi, Kassem Sharif, Ora Shovman, Boris Gilburd, Howard Amital, Yehuda Shoenfeld
BACKGROUND: Anti-glomerular basement membrane (GBM) antibody disease, or Goodpasture's disease, is the clinical manifestation of the production of anti-GBM antibodies, which causes rapidly progressive glomerulonephritis with or without pulmonary hemorrhage. Anti-GBM antibody detection is mandatory for the diagnosis of Goodpasture's disease either from the serum or kidney biopsy. Renal biopsy is necessary for disease confirmation; however, in cases in which renal biopsy is not possible or is delayed, serum detection of anti-GBM antibody is the only way for diagnosis...
July 2017: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/28790860/the-level-of-urinary-semaphorin3a-is-associated-with-disease-activity-in-patients-with-minimal-change-nephrotic-syndrome
#6
Akiko Inoue-Torii, Shinji Kitamura, Jun Wada, Kenji Tsuji, Hirofumi Makino
Semaphorin3A is a secreted protein known to be involved in organogenesis, immune responses and cancer. In the kidney, semaphorin3A is expressed in the glomerular podocytes, distal tubules and collecting tubules, and believed to play a role in the regulation of the kidney development and function. We examined the serum and urinary semaphorin3A levels in 72 patients with renal disease and 5 healthy volunteers. The patients had been diagnosed with thin basement membrane disease (n=4), minimal change nephrotic syndrome (MCNS; n=22), IgA nephritis (n=21), membranous nephropathy (n=16) and focal segmental glomerular sclerosis (n=9)...
2017: International Journal of Nephrology and Renovascular Disease
https://www.readbyqxmd.com/read/28781371/t-helper-type-17-cells-in-immune-mediated-glomerular-disease
#7
REVIEW
Christian F Krebs, Tilman Schmidt, Jan-Hendrik Riedel, Ulf Panzer
CD4(+) T cells are important drivers of tissue damage in immune-mediated renal diseases, such as anti-glomerular basement membrane glomerulonephritis, anti-neutrophil cytoplasmic antibody-associated glomerulonephritis, and lupus nephritis. The discovery of a distinct, IL-17-producing CD4(+) T-cell lineage termed T helper type 17 (TH17) cells has markedly advanced current understanding of the pathogenic mechanisms of organ-specific immunity and the pathways that lead to target organ damage. TH17 cells are characterized by the expression of the transcription factor RORγt, the production of the pro-inflammatory cytokines IL-17A, IL-17F, IL-22, and high expression of the chemokine receptor C-C-motif chemokine receptor 6 (CCR6)...
August 7, 2017: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/28775003/interference-with-gs%C3%AE-coupled-receptor-signaling-in-renin-producing-cells-leads-to-renal-endothelial-damage
#8
Peter Lachmann, Linda Hickmann, Anne Steglich, Moath Al-Mekhlafi, Michael Gerlach, Niels Jetschin, Steffen Jahn, Brigitte Hamann, Monika Wnuk, Kirsten Madsen, Valentin Djonov, Min Chen, Lee S Weinstein, Bernd Hohenstein, Christian P M Hugo, Vladimir T Todorov
Intracellular cAMP, the production of which is catalyzed by the α-subunit of the stimulatory G protein (Gsα), controls renin synthesis and release by juxtaglomerular (JG) cells of the kidney, but may also have relevance for the physiologic integrity of the kidney. To investigate this possibility, we generated mice with inducible knockout of Gsα in JG cells and monitored them for 6 months after induction at 6 weeks of age. The knockout mapped exclusively to the JG cells of the Gsα-deficient animals. Progressive albuminuria occurred in Gsα-deficient mice...
August 3, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28765179/status-epilepticus-as-the-initial-presentation-of-antibody-negative-goodpasture-s-syndrome
#9
Ingrid Pl Ting, Sanihah Abdul Halim, Azreen Adnan, Hasnan Jaafar
Goodpasture's syndrome is a rare pulmonary-renal disease. It is characterised by presence of auto-antibodies directed against the glomerular basement membrane (GBM) antigen. These antibodies that bind to the GBM antigens cause rapidly progressive glomerulonephritis. The alveolar basement membrane also contains similar antigen, leading to pulmonary haemorrhage in active disease. We report a case of a young man who initially presented with status epilepticus and later was found to have rapidly progressive glomerulonephritis with pulmonary haemorrhage...
August 1, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28761240/circulating-monoclonal-igg1-kappa-antibodies-causing-anti-glomerular-basement-membrane-nephritis
#10
M Vankalakunti, R Nada, A Kumar, K Patro, S Ramakrishnan, D Rangarajan
Anti-glomerular basement membrane (GBM) antibody disease is a rare but well-characterized cause of glomerulonephritis. Patients present with rapidly progressive renal failure with hemoptysis. Early diagnosis is crucial in salvaging the renal damage and life-threatening pulmonary hemorrhage. Plasmapheresis and immunosuppression is the mode of therapy. Anti-GBM antibodies are polyclonal in nature. However, rare monoclonal antibodies can cause similar destruction of glomerular capillary walls. We describe distinct combination of circulating monoclonal and anti-GBM nephritis...
July 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28754557/accelerated-podocyte-detachment-and-progressive%C3%A2-podocyte-loss-from-glomeruli-with%C3%A2-age%C3%A2-in-alport-syndrome
#11
Fangrui Ding, Larysa Wickman, Su Q Wang, Yanqin Zhang, Fang Wang, Farsad Afshinnia, Jeffrey Hodgin, Jie Ding, Roger C Wiggins
Podocyte depletion is a common mechanism driving progression in glomerular diseases. Alport Syndrome glomerulopathy, caused by defective α3α4α5 (IV) collagen heterotrimer production by podocytes, is associated with an increased rate of podocyte detachment detectable in urine and reduced glomerular podocyte number suggesting that defective podocyte adherence to the glomerular basement membrane might play a role in driving progression. Here a genetically phenotyped Alport Syndrome cohort of 95 individuals [urine study] and 41 archived biopsies [biopsy study] were used to test this hypothesis...
July 26, 2017: Kidney International
https://www.readbyqxmd.com/read/28748900/dense-deposit-disease-in-a-child-with-febrile-sore-throat
#12
Giovanni Conti, Dominique De Vivo, Agata Vitale, Carmelo Fede, Domenico Santoro
Dense deposit disease or membranoproliferative glomerulonephritis type II is a rare glomerulopathy characterized on renal biopsy by deposition of abnormal electron-dense material in the glomerular basement membrane. The pathophysiologic basis is uncontrolled systemic activation of the alternate pathway of the complement cascade. C3 nephritic factor, an autoantibody directed against the C3 convertase of the alternate pathway, plays a key role. In some patients, complement gene mutations have been identified...
July 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28748894/collagenofibrotic-collagen-type-iii-glomerulopathy-in-association-with-diabetic-nephropathy
#13
Khaled O Alsaad, Burhan Edrees, Khawla A Rahim, Abdulkareem Alanazi, Muawia Ahmad, Noura Aloudah
Collagenofìbrotic (collagen type III) glomerulopathy (CG) is a rare nonimmune-mediated glomerular disease. It is characterized by massive deposition of organized collagen type III fibers, which is localized in the mesangial and subendothelial glomerular areas and associated with increased serum levels of procollagen type III peptide. Association with systemic diseases and malignancies is extremely rare. Herein, we present a case of a nine-year-old girl, known case of type I diabetes mellitus, who presented with fever, nephrotic range proteinuria, generalized edema, and hypertension...
July 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28748185/novel-actions-of-growth-hormone-in-podocytes-implications-for-diabetic-nephropathy
#14
REVIEW
Dhanunjay Mukhi, Rajkishor Nishad, Ram K Menon, Anil Kumar Pasupulati
The kidney regulates water, electrolyte, and acid-base balance and thus maintains body homeostasis. The kidney's potential to ensure ultrafiltered and almost protein-free urine is compromised in various metabolic and hormonal disorders such as diabetes mellitus (DM). Diabetic nephropathy (DN) accounts for ~20-40% of mortality in DM. Proteinuria, a hallmark of renal glomerular diseases, indicates injury to the glomerular filtration barrier (GFB). The GFB is composed of glomerular endothelium, basement membrane, and podocytes...
2017: Frontiers in Medicine
https://www.readbyqxmd.com/read/28747141/biochemical-and-pathological-changes-in-the-male-rat-kidney-and-bladder-following-exposure-to-continuous-900-mhz-electromagnetic-field-on-postnatal-days-22-59
#15
Sibel Türedi, Gökçen Kerimoğlu, Tolga Mercantepe, Ersan Odacı
PURPOSE: To investigate the effect on male rat kidney and bladder tissues of exposure to 900-megahertz (MHz) electromagnetic field (EMF) applied on postnatal days 22-59, inclusive. MATERIALS AND METHODS: Twenty-four male Sprague Dawley rats, aged 21 days, were used. These were divided equally into one of three groups, control (CG), sham (SG) or EMF (EMFG). CG was not exposed to any procedure. SG rats were kept inside a cage, without being exposed to the effect of EMF, for 1 h a day on postnatal days 22-59, inclusive...
July 27, 2017: International Journal of Radiation Biology
https://www.readbyqxmd.com/read/28743882/hyperoside-pre-treatment-prevents-glomerular-basement-membrane-damage-in-diabetic-nephropathy-by-inhibiting-podocyte-heparanase-expression
#16
Xiaofei An, Lin Zhang, Yanggang Yuan, Bin Wang, Qiuming Yao, Ling Li, Jisheng Zhang, Ming He, Jinan Zhang
Glomerular basement membrane (GBM) damage plays a pivotal role in pathogenesis of albuminuria in diabetic nephropathy (DN). Heparan sulfate (HS) degradation induced by podocyte heparanase is the major cause of GBM thickening and abnormal perm-selectivity. In the present study, we aimed to examine the prophylactic effect of hyperoside on proteinuria development and GBM damage in DN mouse model and the cultured mouse podocytes. Pre-treatment with hyperoside (30 mg/kg/d) for four weeks could significantly decrease albuminuria, prevent GBM damage and oxidative stress in diabetes mellitus (DM) mice...
July 25, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28717939/neonatal-stroke-and-haematuria-answers
#17
Sally Kellett, Mathieu Lemaire, Steven P Miller, Christoph Licht, Grace Yoon, Nomazulu Dlamini, Damien Noone
BACKGROUND: This is a report of an infant born near term with neonatal stroke and haematuria. The renal phenotype, pathogenic genotype and pathological findings on renal biopsy are discussed. CASE-DIAGNOSIS: Prenatal magnetic resonance imaging revealed anomalies which persisted postnatally. Haematuria was detected during follow-up. The posttnatal renal ultrasound scan was normal, and there was no associated proteinuria. A likely pathogenic genetic mutation was detected...
July 17, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28717936/neonatal-stroke-and-haematuria-questions
#18
Sally Kellett, Mathieu Lemaire, Steven P Miller, Christoph Licht, Grace Yoon, Nomazulu Dlamini, Damien Noone
This is a report of an infant born near term with neonatal stroke and haematuria. Changes were noted on foetal magnetic resonance images, and these persisted postnatally. A routine renal ultrasound scan during follow-up detected haematuria with no associated proteinuria. A likely pathogenic genetic mutation was identified. This case highlights a relatively newly discovered cause for hereditary nephropathy affecting the basement membrane, initially affecting the glomerular but later the renal tubular basement membranes...
July 17, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28716013/long-term-renal-survival-of-%C3%AE-3-heavy-chain-deposition-disease-a-case-report
#19
Takayuki Katsuno, Shige Mizuno, Masatsuna Mabuchi, Naotake Tsuboi, Atsushi Komatsuda, Shoichi Maruyama
BACKGROUND: Monoclonal immunoglobulin deposition disease (MIDD) is characterized by the non-amyloid deposition of monoclonal immunoglobulin fragments in the basement membranes. Heavy chain deposition disease (HCDD) is a type of MIDD. HCDD is an extremely rare disease, and only three cases have been reported in Japan up to the present. The prognosis of HCDD is very poor, and optimal treatment has not been established. Only a few cases of HCDD with favorable long-term renal prognosis have been reported to date...
July 17, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28704582/autosomal-dominant-form-of-type-iv-collagen-nephropathy-exists-among-patients-with-hereditary-nephritis-difficult-to-diagnose-clinicopathologically
#20
Aya Imafuku, Kandai Nozu, Naoki Sawa, Eiko Hasegawa, Rikako Hiramatsu, Masahiro Kawada, Junichi Hoshino, Kiho Tanaka, Yasuo Ishii, Kenmei Takaichi, Takeshi Fujii, Kenichi Ohashi, Kazumoto Iijima, Yoshifumi Ubara
AIM: Type IV collagen nephropathies include Alport Syndrome and thin basement membrane nephropathy (TBMN), which are caused by mutations in COL4A3/A4/A5 genes. Recently, the report of patients with heterozygous mutations in COL4A3/A4 have been increasing. The clinical course of these patients has a wide variety, and they are diagnosed as TBMN, autosomal dominant Alport syndrome (ADAS), or familial focal segmental glomerular sclerosis. However, diagnosis, frequency and clinicopathological manifestation of them remains unclear...
July 13, 2017: Nephrology
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