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 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 Polycystic Kidney Disease written by Jinghua Hu and published by CRC Press. This book was released on 2019-10-24 with total page 361 pages. Available in PDF, EPUB and Kindle. Book excerpt: This volume focuses on the investigatory methods applied to autosomal dominant polycystic kidney disease (ADPKD), one of the most common human genetic diseases. ADPKD is caused by mutations in PKD1 and TRPP2, two integral membrane proteins that function as receptor/ion channels in primary cilia of tubular epithelial cells. Thus, ADPKD belongs to ciliopathies, a group of disorders caused by abnormal cilia formation or function. This proposed book will cover the state-of-the-art methods ranging from molecular biology, biochemistry, electrophysiology, to tools in model animal studies. Key Features Explores the role of cilia in polycystic kidney disease Focuses on myriad state-of-the-art methods and techniques Reviews specific mutations integral to this autosomal genetic disease Includes discussions of model systems
Download or read book An Investigation Into Endothelial Dysfunction in Patients with Autosomal Dominant Polycystic Kidney Disease written by Adebowale Adekoya and published by . This book was released on 2017 with total page 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 Polycystic Kidney Disease written by Christian Riella and published by Biota Publishing. This book was released on 2017-01-24 with total page 57 pages. Available in PDF, EPUB and Kindle. Book excerpt: This book reviews important aspects of polycystic kidney diseases, the latest scientific understanding of the diseases and syndromes, along with the therapies being developed. Cystic kidney diseases comprise a spectrum of genetic syndromes defined by renal cyst formation and expansion with variable extrarenal manifestations. The most prevalent disorder is the autosomal dominant polycystic kidney disease (ADPKD). It is the most common monogenetic disorder in humans and accounts for 4.4% of end-stage renal disease (ESRD) cases in the U.S. Patients inevitably progress to ESRD and require renal replacement therapy in the form of dialysis or transplantation. Through advancements in genomics and proteomics approaches, novel genes responsible for cystic diseases have been identified, further expanding our understanding of basic mechanisms of disease pathogenesis. The hallmark among all cystic genetic syndromes is the formation and growth of fluid-filled cysts, which originate from tubular epithelia of nephron segments. Cysts are the disease, and treatment strategies are being developed to target prevention or delay of cyst formation and expansion at an early stage, however no such therapy is currently approved.
Download or read book Investigation of a Mouse Model of Autosomal Dominant Polycystic Kidney Disease ADPKD Generated by Targeted Disruption of the Pkd1 Gene written by Sharon Elizabeth Mulroy and published by . This book was released on 2005 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 Pediatric Kidney Disease written by Denis F. Geary and published by Springer. This book was released on 2017-02-16 with total page 1968 pages. Available in PDF, EPUB and Kindle. Book excerpt: The new edition of this valuable clinical resource offers a state of the art, comprehensive review on every clinical condition encountered in pediatric nephrology. International experts present the latest knowledge on epidemiology, diagnosis, management, and prognosis in one concise, clinically focused text, in which care has been taken to couple just the right amount of "need-to-know" basic science with practical clinical guidance that will enable the reader to make efficient, informed decisions. The topics covered include: disorders of renal development, glomerular disorders, the kidney and systemic disease, renal tubular disorders, tubulointerstitial disease, urinary tract disorders, acute kidney injury, hypertension, chronic and end-stage renal disease, and renal replacement therapy. The full-color, highly visual, meticulously crafted format will ensure that the practitioner is able to source and apply information with remarkable ease.
Download or read book Nephrology and Public Health Worldwide written by G.B. Silva Junior and published by Karger Medical and Scientific Publishers. This book was released on 2021-11-02 with total page 369 pages. Available in PDF, EPUB and Kindle. Book excerpt: Nephrology is one of the fastest growing specialties in medicine. Nevertheless, kidney disease is one of the most serious unmet health needs in many countries. To provide healthcare access with the desirable equity worldwide, the nephrology community needs to discuss this public health issue and take part in decisions for elaboration of public health policies with more justice and equity. This book brings together key current public health problems that affect kidney function and illuminates them in contributions by an international group of nephrologists and general practitioners. The chapters review current knowledge and provide guidelines to manage these conditions and decrease the disease burden. At the end, developments in the digital era and their application to kidney disease treatment are synthesized, and a broader outlook on the future of nephrology is given. Ultimately, the publication aims to gather nephrology and public health expertise from researchers from all over the world, providing a broad vision of issues that must be discussed and overcome to guarantee a better treatment for patients with kidney diseases in the world today.
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.
Download or read book Medifocus Guidebook on written by Medifocus.com, Inc. Staff and published by Medifocus_com Inc. This book was released on 2011 with total page 127 pages. Available in PDF, EPUB and Kindle. Book excerpt: The MediFocus Guidebook on Polycystic Kidney Disease is the most comprehensive, up-to-date source of information available. You will get answers to your questions, including risk factors of Polycystic Kidney Disease, standard and alternative treatment options, leading doctors, hospitals and medical centers that specialize in Polycystic Kidney Disease, results of the latest clinical trials, support groups and additional resources, and promising new treatments on the horizon. This one of a kind Guidebook offers answers to your critical health questions including the latest treatments, clinical trials, and expert research; high quality, professional level information you can trust and understand culled from the latest peer-reviewed journals; and a unique resource to find leading experts, institutions, and support organizations including contact information and hyperlinks. This Guidebook was updated on January 18, 2012.
Download or read book A Clinical Sonographic and Genetic Linkage Study of Autosomal Dominant Polycystic Kidney Disease written by David Ravine and published by . This book was released on 1994 with total page 266 pages. Available in PDF, EPUB and Kindle. Book excerpt:
Download or read book The Liver written by Irwin M. Arias and published by John Wiley & Sons. This book was released on 2020-03-09 with total page 1156 pages. Available in PDF, EPUB and Kindle. Book excerpt: Bridging the gap between basic scientific advances and the understanding of liver disease — the extensively revised new edition of the premier text in the field. The latest edition of The Liver: Biology and Pathobiology remains a definitive volume in the field of hepatology, relating advances in biomedical sciences and engineering to understanding of liver structure, function, and disease pathology and treatment. Contributions from leading researchers examine the cell biology of the liver, the pathobiology of liver disease, the liver’s growth, regeneration, metabolic functions, and more. Now in its sixth edition, this classic text has been exhaustively revised to reflect new discoveries in biology and their influence on diagnosing, managing, and preventing liver disease. Seventy new chapters — including substantial original sections on liver cancer and groundbreaking advances that will have significant impact on hepatology — provide comprehensive, fully up-to-date coverage of both the current state and future direction of hepatology. Topics include liver RNA structure and function, gene editing, single-cell and single-molecule genomic analyses, the molecular biology of hepatitis, drug interactions and engineered drug design, and liver disease mechanisms and therapies. Edited by globally-recognized experts in the field, this authoritative volume: Relates molecular physiology to understanding disease pathology and treatment Links the science and pathology of the liver to practical clinical applications Features 16 new “Horizons” chapters that explore new and emerging science and technology Includes plentiful full-color illustrations and figures The Liver: Biology and Pathobiology, Sixth Edition is an indispensable resource for practicing and trainee hepatologists, gastroenterologists, hepatobiliary and liver transplant surgeons, and researchers and scientists in areas including hepatology, cell and molecular biology, virology, and drug metabolism.
Download or read book Oxford Handbook of Clinical Pathology written by James Carton and published by Oxford University Press. This book was released on 2017-08-31 with total page 422 pages. Available in PDF, EPUB and Kindle. Book excerpt: Now in its second edition, the Oxford Handbook of Clinical Pathology is the concise and rapidly accessible guide to general pathologic principles, and the pathology behind common and important conditions across all the major medical and surgical specialties. Fully updated and now including a brand new chapter on head and neck pathology, this book continues to be the definitive resource for medical students and doctors with an interest in this important specialty. Authored by leading experts, The Oxford Handbook of Clinical Pathology uses the best-selling Oxford Handbook format to cover macroscopic and microscopic features alongside immunohistochemical and molecular pathology in a succinct and practical style. This edition is fully illustrated and now includes over new 50 full colour images and staging information for major malignancies to aid the reader and to explain the pathology of common conditions. This handbook continues to be an indispensable resource in the field for medical students, trainees, and biomedical scientists.
Download or read book Nutritional Treatment of Chronic Renal Failure written by Sergio Giovannetti and published by Springer Science & Business Media. This book was released on 2012-12-06 with total page 384 pages. Available in PDF, EPUB and Kindle. Book excerpt: Enormous progress has been made in the treatment of chronic renal failure over the last decades. Until the 1950s, chronic renal failure was considered to be an inexorably lethal condition. This is no longer the case. In addition, the disease, severe uremic syndrome, is now extremely rare, if existent at all, in industrialized countries. Physicians of my generation who saw patients hospitalized with hemor raghes, pericarditis, severe anemia, cardiac failure, "malignant hypertension," pruritus, vomiting, generalized edema, and convulsions are particularly grate ful for this progress. I well remember seeing such patients hospitalized in the last days or weeks of their lives and also remember the sense of impotence I suffered for the com plete lack of efficient measures I had at my disposal to manage their condition. Nowadays, hemodialysis, peritoneal dialysis, and kidney transplantation allow patients with chronic renal failure to survive for very long periods of time in a satisfactory condition. Why then is there still a sense of dissatisfaction and why should we study dietary management? The drawbacks of dialysis and transplantation are the main reasons, but the certainty that dietary therapy is complementary to dialysis and even better than dialysis in certain conditions, is also very important.
