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https://www.readbyqxmd.com/read/28344312/a-case-of-anti-glomerular-basement-membrane-glomerulonephritis-complicated-by-type-1-diabetes-mellitus-mimicking-urinary-tract-infection
#1
Yoshihiro Aoki, Izumi Tanimoto, Yoshihiro Miyauchi, Yoshio Suzuki, Toshiaki Shiojiri
BACKGROUND Type 1 diabetes mellitus (DM) tends to complicate other autoimmune diseases. When considering renal dysfunction in patients with DM, diabetic nephropathy is a likely diagnosis. By contrast, anti-glomerular basement membrane (GBM) glomerulonephritis, an autoimmune disease, is one cause of rapidly progressive glomerulonephritis. CASE REPORT We report the case of a 44-year-old woman diagnosed with anti-glomerular basement membrane (GBM) glomerulonephritis. The diagnosis was made on the basis of serological test results and pathological findings of a renal biopsy...
March 27, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28339567/role-of-biomechanical-forces-in-hyperfiltration-mediated-glomerular-injury-in-congenital-anomalies-of-the-kidney-and-urinary-tract
#2
Tarak Srivastava, Ganesh Thiagarajan, Uri S Alon, Ram Sharma, Ashraf El-Meanawy, Ellen T McCarthy, Virginia J Savin, Mukut Sharma
Congenital anomalies of the kidney and urinary tract (CAKUT) including solitary kidney constitute the main cause of progressive chronic kidney disease (CKD) in children. Children born with CAKUT develop signs of CKD only during adolescence and do not respond to renin-angiotensin-aldosterone system blockers. Early cellular changes underlying CKD progression to end-stage renal disease by early adulthood are not well understood. The mechanism of maladaptive hyperfiltration that occurs from loss of functional nephrons, including solitary kidney, is not clear...
January 17, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28339051/protective-effects-of-tacrolimus-on-podocytes-in-early-diabetic-nephropathy-in-rats
#3
Tao Peng, Xiangdi Chang, Jie Wang, Junhui Zhen, Xiangdong Yang, Zhao Hu
The aim of the present study was to investigate the protective effect of tacrolimus on early podocyte damage in rats with diabetic nephropathy (DN). A total of 38 normal male Sprague‑Dawley rats were randomly divided into four groups: Normal control group (group N; n=8), DN group (n=10), tacrolimus (FK506) treatment group (group F; n=10), benazepril (Lotensin) treatment group (group L; n=10). The rats in groups DN, F and L were administered with streptozotocin (STZ; 60 mg/kg) by intraperitoneal injection to establish the diabetic rat model...
March 22, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28334007/a-functional-variant-in-neph3-gene-confers-high-risk-of-renal-failure-in-primary-hematuric-glomerulopathies-evidence-for-predisposition-to-microalbuminuria-in-the-general-population
#4
Konstantinos Voskarides, Charalambos Stefanou, Myrtani Pieri, Panayiota Demosthenous, Kyriakos Felekkis, Maria Arsali, Yiannis Athanasiou, Dimitris Xydakis, Kostas Stylianou, Eugenios Daphnis, Giorgos Goulielmos, Petros Loizou, Judith Savige, Martin Höhne, Linus A Völker, Thomas Benzing, Patrick H Maxwell, Daniel P Gale, Mathias Gorski, Carsten Böger, Barbara Kollerits, Florian Kronenberg, Bernhard Paulweber, Michalis Zavros, Alkis Pierides, Constantinos Deltas
BACKGROUND: Recent data emphasize that thin basement membrane nephropathy (TBMN) should not be viewed as a form of benign familial hematuria since chronic renal failure (CRF) and even end-stage renal disease (ESRD), is a possible development for a subset of patients on long-term follow-up, through the onset of focal and segmental glomerulosclerosis (FSGS). We hypothesize that genetic modifiers may explain this variability of symptoms. METHODS: We looked in silico for potentially deleterious functional SNPs, using very strict criteria, in all the genes significantly expressed in the slit diaphragm (SD)...
2017: PloS One
https://www.readbyqxmd.com/read/28331448/antibody-mediated-rejection-a-review
#5
Jorge Carlos Garces, Sixto Giusti, Catherine Staffeld-Coit, Humberto Bohorquez, Ari J Cohen, George E Loss
BACKGROUND: Chronic antibody injury is a serious threat to allograft outcomes and is therefore the center of active research. In the continuum of allograft rejection, the development of antibodies plays a critical role. In recent years, an increased recognition of molecular and histologic changes has provided a better understanding of antibody-mediated rejection (AMR), as well as potential therapeutic interventions. However, several pathways are still unknown, which accounts for the lack of efficacy of some of the currently available agents that are used to treat rejection...
2017: Ochsner Journal
https://www.readbyqxmd.com/read/28326083/the-small-tellurium-compound-as101-ameliorates-rat-crescentic-glomerulonephritis-association-with-inhibition-of-macrophage-caspase-1-activity-via-very-late-antigen-4-inactivation
#6
Yafit Hachmo, Yona Kalechman, Itai Skornick, Uzi Gafter, Rachel R Caspi, Benjamin Sredni
Crescentic glomerulonephritis (CGN) is the most aggressive form of GN and, if untreated, patients can progress to end-stage renal failure within weeks of presentation. The α4β1 integrin very late antigen-4 (VLA-4) is an adhesion molecule of fundamental importance to the recruitment of leukocytes in inflammation. We addressed the role of VLA-4 in mediating progressive renal injury in a rat model of CGN using a small tellurium compound. AS101 [ammonium trichloro(dioxoethylene-o,o')tellurate]. This compound has been previously shown to uniquely inhibit VLA-4 activity by redox inactivation of adjacent thiols in the exofacial domain of VLA-4...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28314809/goodpasture-s-disease-with-late-presentation-of-renal-abnormality-and-anti-gbm-autoantibody
#7
Emily Ann Boardman, Sameira Sohail, Rajesh Yadavilli
Goodpasture's disease without circulating autoantibodies is a rare presentation of a rare diagnosis. We present the case of a man aged 17 years who had 3 hospital admissions over a 2-month period with haemoptysis and shortness of breath. Throughout his first 2 admissions, his renal function was normal and anti-glomerular basement membrane (GBM) antibodies were negative. CT pulmonary angiogram during his second admission revealed florid and diffuse alveolar infiltrates. However, high-resolution CT chest performed 4 weeks later showed complete resolution of these changes...
March 17, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28296059/the-pathogenicity-of-t-cell-epitopes-on-human-goodpasture-antigen-and-its-critical-amino-acid-motif
#8
Shui-Yi Hu, Qiu-Hua Gu, Jia Wang, Miao Wang, Xiao-Yu Jia, Zhao Cui, Ming-Hui Zhao
Goodpasture antigen, the non-collagenous domain of α3 chain of type IV collagen [α3(IV)NC1], is the target antigen of anti-glomerular basement membrane (GBM) antibodies. The pathogenicity of T cell epitopes is not elucidated clearly. In this study, we aim to define the nephritogenic T cell epitopes and its critical amino acid residues. Twenty-four overlapping linear peptides were synthesized covering the whole sequence of human α3(IV)NC1. Wistar-Kyoto rats were immunized with linear peptides, and experimental autoimmune glomerulonephritis was evaluated...
March 10, 2017: Journal of Cellular and Molecular Medicine
https://www.readbyqxmd.com/read/28274928/effects-of-childbirth-on-podocyturia-in-women-with-normotensive-uncomplicated-pregnancies
#9
Itsuko Furuta, Tianyue Zhai, Takeshi Umazume, Satoshi Ishikawa, Kinuko Nakagawa, Takashi Kojima, Takahiro Yamada, Mamoru Morikawa, Hisanori Minakami
Changes in hemodynamics and blood pressure occur shortly before and after childbirth regardless of the mode of delivery. This study aimed to test the hypothesis that parturition induces a temporal increase in podocyturia monitored by podocyte-specific protein podocin mRNA expression levels (Pod-mRNA). A total of 105 urine specimens, consisting of 43 and 62 from 18 and 20 otherwise healthy women with vaginal (VD) and elective cesarean deliveries (ECS), respectively, were studied. Determination of urine protein and creatinine concentrations and quantitative analyses of Pod-mRNA, nephrin mRNA (Nep-mRNA), synaptopodin mRNA (Syn-mRNA), and aquaporin 2 mRNA expression (AQP2-mRNA) were performed using RT-PCR in pelleted urine samples...
March 8, 2017: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/28261931/fever-and-prodromal-infections-in-anti-glomerular-basement-membrane-disease
#10
Qiu-Hua Gu, Li-Jun Xie, Xiao-Yu Jia, Rui Ma, Yun-Hua Liao, Zhao Cui, Ming-Hui Zhao
AIM: Anti-glomerular basement membrane (GBM) disease is an autoimmune disorder with rapidly progressive glomerulonephritis and alveolar hemorrhage. Fever symptom and prodromal infections have been reported in many cases, but still not been elucidated. METHODS: Our study enrolled 140 consecutive patients with anti-GBM disease and retrospectively analyzed the characteristics of fever symptom and the possible reasons. RESULTS: Among the 140 patients, 94 (67...
March 6, 2017: Nephrology
https://www.readbyqxmd.com/read/28252672/acute-podocyte-injury-is-not-a-stimulus-for-podocytes-to-migrate-along-the-glomerular-basement-membrane-in-zebrafish-larvae
#11
Florian Siegerist, Antje Blumenthal, Weibin Zhou, Karlhans Endlich, Nicole Endlich
Podocytes have a unique 3D structure of major and interdigitating foot processes which is the prerequisite for renal blood filtration. Loss of podocytes leads to chronic kidney disease ending in end stage renal disease. Until now, the question if podocytes can be replaced by immigration of cells along the glomerular basement membrane (GBM) is under debate. We recently showed that in contrast to former theories, podocytes are stationary in the zebrafish pronephros and neither migrate nor change their branching pattern of major processes over 23 hours...
March 2, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28246329/permeation-of-macromolecules-into-the-renal-glomerular-basement-membrane-and-capture-by-the-tubules
#12
Marlon G Lawrence, Michael K Altenburg, Ryan Sanford, Julian D Willett, Benjamin Bleasdale, Byron Ballou, Jennifer Wilder, Feng Li, Jeffrey H Miner, Ulla B Berg, Oliver Smithies
How the kidney prevents urinary excretion of plasma proteins continues to be debated. Here, using unfixed whole-mount mouse kidneys, we show that fluorescent-tagged proteins and neutral dextrans permeate into the glomerular basement membrane (GBM), in general agreement with Ogston's 1958 equation describing how permeation into gels is related to molecular size. Electron-microscopic analyses of kidneys fixed seconds to hours after injecting gold-tagged albumin, negatively charged gold nanoparticles, and stable oligoclusters of gold nanoparticles show that permeation into the lamina densa of the GBM is size-sensitive...
March 14, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28245485/female-patient-with-alport-syndrome-and-concomitant-membranous-nephropathy-susceptibility-or-association-of-two-diseases
#13
Mariana P Veloso, Precil D M M Neves, Lectícia B Jorge, Cristiane B Dias, Luis Yu, Rafaela B B Pinheiro, Leonardo A Testagrossa, Denise M Malheiros, Bruno E P Balbo, Antônio M Lerário, Luiz F Onuchic, Viktoria Woronik
Alport syndrome (AS) is a disorder of collagen IV, a component of glomerular basement membrane (GBM). The association of AS and immunocomplex nephropathies is uncommon. This is a case of a 37-year-old woman with family history of X-linked AS, including 4 affected sons. This patient developed full-blown nephrotic syndrome along a 3-month period, a presentation not consistent with AS progression. This scenario suggested an alternative diagnosis. A kidney biopsy was therefore performed, showing membranous nephropathy (MN) in addition to GBM structural alterations compatible with AS...
March 1, 2017: Nephron
https://www.readbyqxmd.com/read/28237245/the-formation-of-quiescent-glomerular-endothelial-cell-monolayer-in-vitro-is-strongly-dependent-on-the-choice-of-extracellular-matrix-coating
#14
Kamilla Pajęcka, Malik Nygaard Nielsen, Troels Krarup Hansen, Julie M Williams
BACKGROUND AND AIMS: Nephropathy involves pathophysiological changes to the glomerulus. The primary glomerular endothelial cells (GEnCs) have emerged as an important tool for studying glomerulosclerotic mechanisms and in the screening process for drug-candidates. The success of the studies is dependent on the quality of the cell model. Therefore, we set out to establish an easy, reproducible model of the quiescent endothelial monolayer with the use of commercially available extracellular matrices (ECMs)...
February 22, 2017: Experimental Cell Research
https://www.readbyqxmd.com/read/28236514/familial-hematuria-a-review
#15
REVIEW
Pavlína Plevová, Josef Gut, Jan Janda
The most frequent cause of familial glomerular hematuria is thin basement membrane nephropathy (TBMN) caused by germline COL4A3 or COL4A4 gene mutations. Less frequent but important cause with respect to morbidity is Alport syndrome caused by germline COL4A5 gene mutations. The features of Alport syndrome include hematuria, proteinuria and all males with X-linked disease and all individuals with recessive disease will develop end stage renal disease, usually at early youth. In X-linked Alport syndrome, a clear genotype-phenotype correlation is typically observed in men...
January 31, 2017: Medicina
https://www.readbyqxmd.com/read/28228399/accumulation-of-worn-out-gbm-material-substantially-contributes-to-mesangial-matrix-expansion-in-diabetic-nephropathy
#16
Wilhelm Kriz, Jana Löwen, Giuseppina Federico, Jacob van den Born, Elisabeth Gröne, Hermann-Josef Gröne
Thickening of the glomerular basement membrane (GBM) and expansion of the mesangial matrix are hallmarks of diabetic nephropathy (DN) generally considered to emerge from different sites of overproduction; GBM components from podocytes and mesangial matrix from mesangial cells. Re-evaluation of 918 biopsies with DN revealed strong evidence that these mechanisms are connected to each other, wherein excess GBM components fail to undergo degradation and are deposited in the mesangium. These data do not exclude that also mesangial cells synthesize components that contribute to the accumulation of matrix in the mesangium...
February 22, 2017: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/28219886/dependence-of-glomerulonephritis-induction-on-novel-intraglomerular-alternatively-activated-bone-marrow-derived-macrophages-and-mac-1-and-pd-l1-in-lupus-prone-nzm2328-mice
#17
Sun-Sang J Sung, Yan Ge, Chao Dai, Hongyang Wang, Shu Man Fu, Rahul Sharma, Young S Hahn, Jing Yu, Thu H Le, Mark D Okusa, Warren K Bolton, Jessica R Lawler
Glomerular damage mediated by glomerulus-infiltrating myeloid-derived cells is a key pathogenic event in lupus nephritis (LN), but the process is poorly understood. Confocal microscopy of kidney sections and flow cytometry analysis of glomerular cells from magnetic bead-purified glomeruli have identified glomerulus-infiltrating leukocyte populations in NZM2328 (NZM) lupus-prone mice with spontaneous chronic glomerulonephritis (GN) and anti-glomerular basement membrane-induced nephritis. The occurrence of a major glomerulus-infiltrating CD11b(+)F4/80(-)I-A(-) macrophage population exhibiting the markers programmed death ligand-1 (PD-L1), Mac-2, and macrophage mannose receptor (CD206) and producing Klf4, Il10, Retnla, Tnf, and Il6 mRNA, which are known to be expressed by alternatively activated (M2b) macrophages, correlated with proteinuria status...
April 1, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28212178/the-long-journey-through-renal-filtration-new-pieces-in-the-puzzle-of-slit-diaphragm-architecture
#18
Sara Conti, Luca Perico, Florian Grahammer, Tobias B Huber
PURPOSE OF REVIEW: The podocyte slit diaphragm is probably the least understood component of the kidney filtration barrier. In this review, we aim to integrate the most recent findings on the molecular make-up and structural architecture of this specialized cell-cell junction into a current concept of glomerular filtration. RECENT FINDINGS: Analysis of cryopreserved mammalian tissue revealed a bipartite composition of the slit diaphragm. Single NEPH1 molecules span the lower part of the slit close to the glomerular basement membrane whereas NEPHRIN molecules are positioned in the apical part toward Bowman's space...
February 16, 2017: Current Opinion in Nephrology and Hypertension
https://www.readbyqxmd.com/read/28191821/crucial-role-of-mesangial-cell-derived-connective-tissue-growth-factor-in-a-mouse-model-of-anti-glomerular-basement-membrane-glomerulonephritis
#19
Naohiro Toda, Kiyoshi Mori, Masato Kasahara, Akira Ishii, Kenichi Koga, Shoko Ohno, Keita P Mori, Yukiko Kato, Keisuke Osaki, Takashige Kuwabara, Katsutoshi Kojima, Daisuke Taura, Masakatsu Sone, Taiji Matsusaka, Kazuwa Nakao, Masashi Mukoyama, Motoko Yanagita, Hideki Yokoi
Connective tissue growth factor (CTGF) coordinates the signaling of growth factors and promotes fibrosis. Neonatal death of systemic CTGF knockout (KO) mice has hampered analysis of CTGF in adult renal diseases. We established 3 types of CTGF conditional KO (cKO) mice to investigate a role and source of CTGF in anti-glomerular basement membrane (GBM) glomerulonephritis. Tamoxifen-inducible systemic CTGF (Rosa-CTGF) cKO mice exhibited reduced proteinuria with ameliorated crescent formation and mesangial expansion in anti-GBM nephritis after induction...
February 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28187981/deglycosylation-of-myeloperoxidase-uncovers-its-novel-antigenicity
#20
Jun-Tao Yu, Jian-Nan Li, Jia Wang, Xiao-Yu Jia, Zhao Cui, Ming-Hui Zhao
Myeloperoxidase (MPO) is a common target antigen of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis and is recognized in one-third of patients with anti-glomerular basement membrane (GBM) disease. Our previous study identified over 60% of patients with anti-GBM disease recognizing linear peptides of MPO heavy chain. Here we tested whether aberrant glycosylation alters MPO antigenicity through exposure of neo-epitopes on MPO molecules. Atypical glycosylated MPO molecules, including all possible glycosylation types, were prepared by exoglycosidase and endoglycosidase treatments...
February 7, 2017: Kidney International
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