Download or read book Extracellular Matrix Abnormalities in Polycystic Kidney Disease written by Soundarapandian Vijayakumar and published by . This book was released on 2012 with total page pages. Available in PDF, EPUB and Kindle. Book excerpt: Extracellular Matrix Abnormalities in Polycystic Kidney Disease.
Download or read book Investigating the Pathophysiology of Autosomal Dominant Polycystic Kidney Disease written by Audra Jeanne Charron and published by . This book was released on 1999 with total page pages. Available in PDF, EPUB and Kindle. Book excerpt: Autosomal dominant polycystic kidney disease (ADPKD) is characterized by perturbations in extracellular matrix synthesis, cellular polarity, gene expression, and proliferative potential of the epithelial cells lining renal cysts. The mechanisms whereby these cells express a partially dedifferentiated phenotype have remained enigmatic despite the identification of the causative genetic lesions. A comprehensive assessment of epithelial cell architecture, molecular trafficking, cellular plasticity, and extracellular matrix profile was therefore undertaken. These analyses revealed several anomalies as candidate factors that undoubtedly undermine the integrity of ADPKD tissue. Within explanted ADPKD cells the cytoskeletal networks were biochemically or structurally immature. Moreover, both the adherens junction protein E-cadherin and basolateral targeting patch components were depleted from the basolateral ADPKD cell surface. These derangements correlated with impaired vectorial trafficking: while apical transport proceeded effectively, basolateral trafficking in the diseased cells was inefficient. Stalling of traffic along the basolateral exocytic route was evident as an accumulation of exocytic cargo within the dilated ADPKD cell Golgi and the redistribution of several components of the basolateral trafficking machinery. The architectural and behavioral disturbances in the mutant cells prompted an examination of the interplay between cellular phenotype and extracellular matrix. Deprived of native environmental cues by explantation into monolayer culture, diseased cells were unable to fully redifferentiate. Furthermore, while normal kidney cells formed branching tubules in collagen-I matrices, ADPKD cells assembled spherical cysts or failed altogether to organize into multicellular aggregates. Remarkably, ADPKD cells cultured within purified native extracellular matrix successfully completed branching morphogenesis, demonstrating that genetic lesions did not completely abolish the morphogenetic potential of these cells. The dissimilar behavior of ADPKD cells cultured in different matrices suggested that the extracellular matrix in ADPKD tissue is permissive for cystogenesis. The aberrant deposition of a novel matrix microfibril protein within the basement membrane circumscribing ADPKD cysts represents an environmental cue that likely exacerbates atypical cellular behavior. Considered together, the results presented herein imply the existence of a pathological circuit initiated by the acquisition of genetic mutations and perpetuated by cellular dedifferentiation, ultimately leading to tissue dysmorphogenesis and the degeneration of renal epithelia.
Download or read book Cystogenesis written by Jong Hoon Park and published by Springer. This book was released on 2016-10-12 with total page 128 pages. Available in PDF, EPUB and Kindle. Book excerpt: Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a highly prevalent hereditary renal disorder in which fluid-filled cysts are appeared in both kidneys. Main causative genes of ADPKD are PKD1 and PKD2, encoding for polycystin-1 (PC1) and polycystin-2 (PC2) respectively. Those proteins are localized on primary cilia and function as mechanosensor in response to the fluid flow, translating mechanistic stimuli into calcium signaling. With mutations either of PKD1 or PKD2, hyper-activated renal tubular epithelial cell proliferation is observed, followed by disrupted calcium homeostasis and aberrant intracellular cyclic AMP (cAMP) accumulation. Increased cell proliferation with fluid secretion leads to the development of thousands of epithelial-lined, fluid-filled cysts in kidneys. It is also accompanied by interstitial inflammation, fibrosis, and finally reaching end-stage renal disease (ESRD). In human ADPKD, the age at which renal failure typically occurs is later in life, however no specific targeted medications are available to cure ADPKD. Recently, potential therapeutic targets or surrogate diagnostic biomarkers for ADPKD are proposed with the advances in the understanding of ADPKD pathogenesis, and some of them were attempted for clinical trials. Herein, we will summarize genetic and epi-genetic molecular mechanisms in ADPKD progression, and overview the currently available biomarkers or potential therapeutic reagents suggested.
Download or read book Autosomal Dominant Polycystic Kidney Disease written by Ashraf Nimer Malhas and published by . This book was released on 2004 with total page 520 pages. Available in PDF, EPUB and Kindle. Book excerpt:
Download or read book Advances in the Pathogenesis of Polycystic Kidney Disease written by Frank A. Carone and published by . This book was released on 1990 with total page 194 pages. Available in PDF, EPUB and Kindle. Book excerpt:
Download or read book Silva s Diagnostic Renal Pathology written by Xin J. Zhou and published by Cambridge University Press. This book was released on 2017-03-02 with total page 691 pages. Available in PDF, EPUB and Kindle. Book excerpt: An algorithmic approach to interpreting renal pathology, updated in light of recent advances in understanding and new classification schemes.
Download or read book Pediatric Nephrology written by Ellis D. Avner and published by Springer Science & Business Media. This book was released on 2009-08-20 with total page 2059 pages. Available in PDF, EPUB and Kindle. Book excerpt: Here is an extensive update of Pediatric Nephrology, which has become the standard reference text in the field. It is global in perspective and reflects the international group of editors, who are well-recognized experts in pediatric nephrology. Within this text, the development of kidney structure and function is followed by detailed and comprehensive chapters on all childhood kidney diseases.
Download or read book Extracellular Matrix in the Kidney written by Hikaru Koide and published by . This book was released on 1994 with total page 244 pages. Available in PDF, EPUB and Kindle. Book excerpt:
Download or read book Renal Fibrosis Mechanisms and Therapies written by Bi-Cheng Liu and published by Springer. This book was released on 2019-08-09 with total page 709 pages. Available in PDF, EPUB and Kindle. Book excerpt: This book systemically presents the latest research on renal fibrosis, covering all the major topics in the field, including the possible mechanisms, biomarkers, and strategies for prevention and treatment of chronic kidney disease (CKD). Due to its high prevalence, CKD represents a huge global economic and social burden. Irrespective of the initial causes, CKD progresses to end stage kidney disease (ESKD) due to renal fibrosis, which is characterized by glomerulosclerosis, tubule atrophy and atresia, and the excessive accumulation of extracellular matrix (ECM) in the kidney. Unfortunately, an estimated 1%-2% of the adult population living with CKD will need renal replacement therapy at some point as a result of ESKD. As such, strategies for preventing or slowing CKD progression to ESKD are of utmost importance, and studies aiming to understand the mechanisms of renal fibrosis have been the focus of intensive research. Recently, novel insights into the pathophysiological processes have furthered our understanding of the pathogenesis of renal fibrosis, and more importantly, promoted studies on the early diagnosis and treatment of CKD. This book draws lessons from the extensive, state-of-the-art research in this field, elaborating the new theories and new techniques to offer readers a detailed and comprehensive understanding of renal fibrosis and as well as inspiration for future research directions.
Download or read book Novel Insights on Chronic Kidney Disease Acute Kidney Injury and Polycystic Kidney Disease written by and published by . This book was released on 2012 with total page pages. Available in PDF, EPUB and Kindle. Book excerpt:
Download or read book Chronic Kidney Disease written by Thomas Rath and published by BoD – Books on Demand. This book was released on 2018-02-21 with total page 307 pages. Available in PDF, EPUB and Kindle. Book excerpt: Known worldwide, chronic kidney disease (CKD) is a disease that affects up to 4% of the population with increasing figures also in the developing countries. Life expectancy of patients affected by CKD is shortened compared to the overall population, and only a minority of patients reach end-stage renal disease (ESRD) with the need for dialysis or renal transplantation; death overtakes dialysis. In the 13 chapters, this book sheds light on the different aspects related to pathophysiology and clinical aspects of CKD, providing interesting insights into not only inflammation and cardiovascular risk but also the interplay of hormones and the functional aspects of endothelial function. In addition, chapters dealing with genetic aspects of polycystic kidney disease and also the clinical handling of patients with CKD and peritoneal dialysis will be beneficial for the open-minded reader.
Download or read book The Cystic Kidney written by K.D. Gardner and published by Springer Science & Business Media. This book was released on 2012-12-06 with total page 441 pages. Available in PDF, EPUB and Kindle. Book excerpt: This is a book about renal cysts and cystic kidneys. Its contributors have created a resource of current information in a field that once aroused only curiosity, but that now stands at the leading edge of molecular nephrology. Its authorship includes 'oldtimers', who bring the wisdom of experience, and 'newcomers', whose presence attests to the contributions made by the investigative and technological advances of the past decade. Its text is organized to carry the reader from renal cyst to cystic renal disease. Each of its chapters defines or explores a challenge or an advance. Cells that line renal cysts are diverse in structure, type, and perhaps function. The cysts themselves lie within an interstitium that is not normal and may influence cyst development and growth. Experimental analogs of human disease offer increasing opportunities to basic researchers to examine, in sequence and under controlled circumstances, those events that favor nephron dilation, cyst growth and ultimate renal failure.
Download or read book Kidney Development and Disease written by Rachel K. Miller and published by Springer. This book was released on 2017-04-13 with total page 373 pages. Available in PDF, EPUB and Kindle. Book excerpt: Kidney Development and Disease brings together established and young investigators who are leading authorities in nephrology to describe recent advances in three primary areas of research. The first section describes the use of animal models as powerful tools for the discovery of numerous molecular mechanisms regulating kidney development. The second section focuses on nephric cell renewal and differentiation, which lead to diverse cell fates within the developing kidney, and discusses diseases resulting from the aberrant regulation of the balance between cell fate decisions. The final section concentrates on morphogenesis of the developing kidney and its maintenance after formation as well as the diseases resulting from failures in these processes. Kidney form and function have been extensively studied for centuries, leading to discoveries related to their development and disease. Recent scientific advances in molecular and imaging techniques have broadened our understanding of nephron development and maintenance as well as the diseases related to these processes.
Download or read book Polycystic Kidney Disease written by Benjamin D. Cowley, Jr. and published by Springer. This book was released on 2018-05-24 with total page 274 pages. Available in PDF, EPUB and Kindle. Book excerpt: This comprehensive guide to polycystic kidney disease captures the growing knowledge of this common, potentially-fatal and hereditary disease. The first two sections of the book provide an overview of PKD gene structures, mutations and pathophysiologic mechanisms. This is followed by chapters focused on PKD’s clinical features, including renal and extrarenal manifestations, and appropriate management of patients. The final section covers current clinical trials and emerging therapies in PKD. Authored by experts in the field, this book provides the clinician and researcher with critical information on basic and translational science and clinical approaches in one concise resource.
Download or read book Oxford Textbook of Clinical Nephrology written by Neil N. Turner and published by Oxford University Press. This book was released on 2015-10-29 with total page 3045 pages. Available in PDF, EPUB and Kindle. Book excerpt: This fourth edition of the Oxford Textbook of Clinical Nephrology builds on the success and international reputation of the publication as an important resource for the practising clinician in the field. It provides practical, scholarly, and evidence-based coverage of the full spectrum of clinical nephrology, written by a global faculty of experts. The most relevant and important reference to clinical nephrology, this is an authoritative and comprehensive textbook combining the clinical aspects of renal disease essential to daily clinical practice with extensive information about the underlying basic science and current evidence available. Each section of the textbook has been critically and comprehensively edited under the auspices of a leading expert in the field. This new edition has been significantly expanded and reapportioned to reflect developments and new approaches to topics, and includes treatment algorithms to aid and enhance patient care where possible. The fourth edition offers increased focus on the medical aspects of transplantation, HIV-associated renal disease, and infection and renal disease, alongside entirely new sections on genetic topics and clinical and physiological aspects of fluid/electrolyte and tubular disorders. The emphasis throughout is on marrying advances in scientific research with clinical management. Richly illustrated throughout in full colour, this is a truly modern and attractive edition which reinforces the Oxford Textbook of Clinical Nephrology's position as an indispensable reference work of consistent quality and reliability. Enriched and refined by careful revision, this new edition continues the tradition of excellence. This print edition of The Oxford Textbook of Clinical Nephrology comes with a year's access to the online version on Oxford Medicine Online. By activating your unique access code, you can read and annotate the full text online, follow links from the references to primary research materials, and view, enlarge and download all the figures and tables. Oxford Medicine Online is mobile optimized for access when and where you need it.
Download or read book Liver Disease in Children written by Frederick J. Suchy and published by Cambridge University Press. This book was released on 2021-03-18 with total page 875 pages. Available in PDF, EPUB and Kindle. Book excerpt: Liver disease in children is increasing in prevalence, placing a huge burden on healthcare systems and often requiring long-term management. Offering an integrative approach to the science and clinical practice of pediatric hepatology, this is the definitive reference text for improved diagnosis and treatment strategies. In the new edition of this authoritative text, chapters have been thoroughly revised in line with major advances in the field, such as recognizing the increased frequency of fatty liver disease, and how genetic testing has the potential to establish earlier diagnoses for a variety of diseases. Disorders covered include cholestasis, metabolic disorders and hepatitis, with their presentation across the spectrum of infancy, childhood and adolescence discussed. The indications and surgical aspects of liver transplant are explained and post-transplant care is described in detail. This is a valuable resource for pediatricians, hepatologists, gastroenterologists and all clinicians involved in the care of children with liver diseases.
Download or read book Fas Signaling written by Harald Wajant and published by Springer Science & Business Media. This book was released on 2007-04-03 with total page 169 pages. Available in PDF, EPUB and Kindle. Book excerpt: Fas Signaling is focused on the signaling mechanisms and biology of the prototypic death receptor Fas, also called CD95 or APO-1. The chapters of this book cover, besides the well recognized apoptosis-related functions of Fas, its emerging role as a proinflammatory cytokine and as an inducer of alternative forms of cell death. Fas Signaling aims to provide the reader with an up-to-date survey of the various aspects of Fas biology and the open questions of the field are addressed. This title is intended for Ph.D and post-doctoral students starting to work in the field, but is also useful for everyone with an interest in the biology of this exciting molecule.
