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Glomerular basement membrane

Cristina Zennaro, Maria Pia Rastaldi, Gerald James Bakeine, Riccarda Delfino, Federica Tonon, Rossella Farra, Gabriele Grassi, Mary Artero, Massimo Tormen, Michele Carraro
Although it is well recognized that cell-matrix interactions are based on both molecular and geometrical characteristics, the relationship between specific cell types and the three-dimensional morphology of the surface to which they are attached is poorly understood. This is particularly true for glomerular podocytes - the gatekeepers of glomerular filtration - which completely enwrap the glomerular basement membrane with their primary and secondary ramifications. Nanotechnologies produce biocompatible materials which offer the possibility to build substrates which differ only by topology in order to mimic the spatial organization of diverse basement membranes...
2016: International Journal of Nanomedicine
Shinya Kawamoto, Ryo Koda, Atsunori Yoshino, Tetsuro Takeda, Yoshihiko Ueda
Medullary cystic kidney disease (MCKD) is a hereditary disease associated with bilateral medullary polycysts and interstitial fibrosis. MCKD is typically associated with slowly progressive renal dysfunction. We herein report two rare elderly cases with enlarged kidneys and rapidly progressive renal dysfunction without myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA), PR3-ANCA, or anti-glomerular basement membrane (GBM) antibodies. Renal biopsies revealed extensive tubular dilatation and atrophy with interstitial fibrosis consistent with MCKD...
2016: Internal Medicine
Toshiharu Ueno, Koichi Kikuchi, Ryo Hazue, Koki Mise, Keiichi Sumida, Noriko Hayami, Tatsuya Suwabe, Junichi Hoshino, Naoki Sawa, Kenji Arizono, Shigeko Hara, Kenmei Takaichi, Takeshi Fujii, Kenichi Ohashi, Yoshifumi Ubara
A 58-year-old man was referred to our institution for an evaluation of nephrotic range proteinuria. Renal biopsy showed a marked expansion of the mesangial matrix and thickening of glomerular basement membrane (GBM) in periodic acid-silver methenamine (PAM). Immunofluorescence (IF) revealed strong staining for the monoclonal kappa light chain. EM demonstrated massive subendothelial and mesangial dense deposits. As a result, light chain deposition disease (LCDD) was diagnosed. Melphalan and prednisolone (MP) therapy was started, which was continued for 10 years with minimal complications...
2016: Internal Medicine
Naohiro Toda, Hideki Yokoi, Kiyoshi Mori, Masashi Mukoyama
CCN2 has been shown to be closely involved in the progression of renal fibrosis, indicating the potential of CCN2 inhibition as a therapeutic target. Although the examination of the phenotypes of adult CCN2 knockout mice with renal diseases has yielded valuable scientific insights, perinatal death has limited studies of CCN2 in vivo. Conditional knockout technology has become widely used for the deletion of genes in the desired cell populations and time points through the use of cell-specific Cre recombinase-expressing mice...
2017: Methods in Molecular Biology
Mathews V Noble, S Sajeesh, S Renuka, T Kurian
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
Yunqiang He, Ziqiang Xu, Mingshi Zhou, Minmin Wu, Xuehai Chen, Silu Wang, Kaiyan Qiu, Yong Cai, Hongxing Fu, Bicheng Chen, Mengtao Zhou
Objective. Diabetic nephropathy (DN) is a common microvascular complication of diabetes mellitus, and insulin therapy has many side effects in the treatment of DN. Islet transplantation has emerged as a promising therapy for diabetic patients. This study was established to investigate its advantageous effects in a rat model of early DN. Methods. Streptozotocin was administered to the rats to induce diabetes. Twelve weeks later, the diabetic rats were divided into 3 groups: the islet-transplanted group (IT group), the insulin-treated group (IN group), and the untreated group (DN group)...
2016: Journal of Diabetes Research
Michael J Randles, Sophie Collinson, Tobias Starborg, Aleksandr Mironov, Mira Krendel, Eva Königshausen, Lorenz Sellin, Ian S D Roberts, Karl E Kadler, Jeffrey H Miner, Rachel Lennon
Glomeruli are highly sophisticated filters and glomerular disease is the leading cause of kidney failure. Morphological change in glomerular podocytes and the underlying basement membrane are frequently observed in disease, irrespective of the underlying molecular etiology. Standard electron microscopy techniques have enabled the identification and classification of glomerular diseases based on two-dimensional information, however complex three-dimensional ultrastructural relationships between cells and their extracellular matrix cannot be easily resolved with this approach...
October 11, 2016: Scientific Reports
Yi Zhou, Bao-Ying Li, Xiao-Li Li, Ya-Juan Wang, Zhen Zhang, Fei Pei, Quan-Zhen Wang, Jun Zhang, Ya-Wei Cai, Mei Cheng, Hai-Qing Gao
Grape seed procyanidin B2 (GSPB2) exerts a variety of potent protective pharmacological effects on diabetic complications. The renal protective effects of GSPB2 and the target protein mimecan regulated by GSPB2, discovered in a previous quantitative proteomic analysis, were assessed in mice with diabetic nephropathy Twenty-four db/db mice were divided into 2 groups of the vehicle-treated and GSPB2-treated (30 mg/kg/d) diabetic groups. All animals were observed for 10 weeks. Treatment with GSPB2 resulted in an improvement in body weight increase and serum levels of triglyceride, total cholesterol, advanced glycation end products, and urinary albumin excretion in comparison with the vehicle-treated diabetic mice (P < ...
September 2016: Iranian Journal of Kidney Diseases
Laura Penna Rocha, Samuel Cavalcante Xavier, Fernanda Rodrigues Helmo, Juliana Reis Machado, Fernando Silva Ramalho, Marlene Antônia Dos Reis, Rosana Rosa Miranda Corrêa
INTRODUCTION: Epithelial-mesenchymal transition (EMT) is a process in which epithelial cells may express mesenchymal cell markers with subsequent change in their functions, and it may be part of the etiopathogenesis of kidney disease. OBJECTIVE: The aim of this study was to evaluate the immunexpression of some EMT inducers and markers in frequent nephropathies in pediatric patients. METHODS: 59 patients aged 2-18 years old were selected and divided into 6 groups of frequent nephropathies in children and adolescents, as well as one control group...
September 22, 2016: Pathology, Research and Practice
Longquan Xu, Sylvia Hiller, Stephen Simington, Volker Nickeleit, Nobuyo Maeda, Leighton R James, Xianwen Yi
Oxidative stress is implicated in the pathogenesis of diabetic nephropathy (DN) but outcomes of many clinical trials are controversial. To define the role of antioxidants in kidney protection during the development of diabetic nephropathy, we have generated a novel genetic antioxidant mouse model with over- or under-expression of lipoic acid synthase gene (Lias). These models have been mated with Ins2Akita/+ mice, a type I diabetic mouse model. We compare the major pathologic changes and oxidative stress status in two new strains of the mice with controls...
2016: PloS One
L Fialová
Autoantibodies directed against various self-antigens comprise a heterogeneous group of immunoglobulins, which differ in their qualitative and quantitative features. An important qualitative characteristic of antibodies is affinity/avidity, which changes in the process of its maturation during the immune response.This study is aimed to summarize the knowledge about avidity of selected autoantibodies in certain autoimmune diseases. The avidity of various autoantibodies differs under distinct clinical situations...
2016: Epidemiologie, Mikrobiologie, Imunologie
John Dixon, Hannah Wilkinson, Kamran Iqbal, Virginia Quan
We present the first known case of pulmonary artery aneurysms as a feature of Recurrent Anti-Glomerular Basement Membrane disease.
September 2016: JRSM Open
Claire Dossier, Agnès Jamin, Georges Deschênes
Steroid sensitive nephrotic syndrome is marked by a massive proteinuria and loss of podocytes foot processes. The mechanism of the disease remains debated but recent publications suggest a primary role of EBV. EBV replication in the peripheral blood is found in 50% of patients during the first flare of the disease. The genetic locus of steroid sensitive nephrotic syndrome was also identified as influencing antibodies directed against EBNA1. EBV is able to establish, latent benign infection in memory B cells that display phenotypes similar to antigen-selected memory B cells...
September 28, 2016: Pediatric Research
Nobuya Yoshida, Denis Comte, Masayuki Mizui, Kotaro Otomo, Florencia Rosetti, Tanya N Mayadas, José C Crispín, Sean J Bradley, Tomohiro Koga, Michihito Kono, Maria P Karampetsou, Vasileios C Kyttaris, Klaus Tenbrock, George C Tsokos
Inducible cAMP early repressor (ICER) has been described as a transcriptional repressor isoform of the cAMP response element modulator (CREM). Here we report that ICER is predominantly expressed in Th17 cells through the IL-6-STAT3 pathway and binds to the Il17a promoter, where it facilitates the accumulation of the canonical enhancer RORγt. In vitro differentiation from naive ICER/CREM-deficient CD4(+) T cells to Th17 cells is impaired but can be rescued by forced overexpression of ICER. Consistent with a role of Th17 cells in autoimmune and inflammatory diseases, ICER/CREM-deficient B6...
2016: Nature Communications
Bobby Gu, Alex B Magil, Sean J Barbour
Anti-glomerular basement membrane (GBM) antibody disease is a typically monophasic autoimmune disease with severe pulmonary and renal involvement. We report an atypical case of frequently relapsing anti-GBM antibody disease with both anti-GBM antibody-positive flares with pulmonary and renal involvement, and anti-GBM antibody-negative flares that were pulmonary limited with no histologic renal disease. This is the first report of alternating disease phenotype and anti-GBM antibody status over time. Disease severity paralleled the detection of anti-GBM antibodies but was independent of IgG subtype staining along the GBM...
October 2016: Clinical Kidney Journal
Patricia Liu, Sana Waheed, Lamya Boujelbane, Laura J Maursetter
Anti-glomerular basement membrane (GBM) disease is commonly a monophasic illness. We present the case of multiple recurrences of anti-GBM disease with varying serum anti-GBM antibody findings. A 33-year-old female tobacco user presenting with hematuria was diagnosed with anti-GBM disease by renal biopsy. Five years later, she presented with alveolar hemorrhage and positive anti-GBM antibody. She presented a third time with alveolar hemorrhage but undetectable anti-GBM antibody. With each occurrence, symptoms resolved with plasmapheresis, intravenous methylprednisone and oral cyclophosphamide...
October 2016: Clinical Kidney Journal
Richard J Glassock
Anti-glomerular basement membrane (GBM) disease usually pursues a self-limited course, at least from the immunological perspective. In addition, circulating antibodies to cryptic, conformational epitopes within the NC1 domain of the alpha 3 chain of Type IV Collagen are commonly found at the zenith of the clinical disease. However, exceptions to these general rules do occur, as exemplified by two remarkable cases reported in this issue of the Clinical Kidney Journal. The possible explanations for and the lessons learned from these uncommon occurrences are discussed in this short commentary...
October 2016: Clinical Kidney Journal
Turki Al Hussain, Hadeel Al Mana, Maged H Hussein, Mohammed Akhtar
The glomerulus has 3 resident cells namely mesangial cells that produce the mesangial matrix, endothelial cells that line the glomerular capillaries, and podocytes that cover the outer surface of the glomerular basement membrane. Parietal epithelial cells (PrECs), which line the Bowman's capsule are not part of the glomerular tuft but may have an important role in the normal function of the glomerulus. A significant progress has been made in recent years regarding our understanding of the role and function of these cells in normal kidney and in kidneys with various types of glomerulopathy...
September 26, 2016: Advances in Anatomic Pathology
Maria G Detsika, Pu Duann, Vassilios Atsaves, Apostolos Papalois, Elias A Lianos
Complement-activation controllers, including decay accelerating factor (DAF), are gaining emphasis as they minimize injury in various dysregulated complement-activation disorders, including glomerulopathies. Heme oxygenase (HO)-1 overexpression or induction has been shown to attenuate injury in complement-dependent models of glomerulonephritis. This study investigated whether up-regulation of DAF by heme oxygenase 1 (HO-1) is an underlying mechanism by using Hmox-1-deficient rats (Hmox1(+/-); Hmox1(-/-)) or rats with Hmox-1 overexpression targeted to glomerular epithelial cells (GEC(Hmox-1)), which are particularly vulnerable to complement-mediated injury owing to their terminally differentiated nature...
September 20, 2016: American Journal of Pathology
Kye Won Kwon, Hyeon Joo Jeong, Jang Han Lee
Podocytic infolding glomerulopathy (PIG) is a rare glomerular abnormality involving glomerular basement membrane (GBM) bubbling viewable by light microscopy, extensive invagination of the podocytic cytoplasm, and the presence of microstructures viewable by electron microscopy. PIG was proposed as a new disease entity in 2008. However, cases have been reported exclusively in Japan and no case reports outside Japan have been published. Here, we report a case of PIG in a 44-year-old Korean female. The patient showed mild proteinuria without renal functional impairment or other systemic diseases...
September 23, 2016: Ultrastructural Pathology
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