The Role of Pendrin in Health and Disease


Book Description

This book reviews the current state of knowledge on the genetics, molecular biology and physiology of pendrin, with a particular focus on pendrin dysfunction and the consequences for human health. Pendrin is a membrane transport protein expressed in the thyroid, inner ear, kidney and airways, and was recently found in a variety of other tissues and organs. Pendrin malfunction may cause a genetic disease called Pendred syndrome or non-syndromic deafness. The book provides a thorough description of the multifaceted role of pendrin in human health and disease. As such, it offers an invaluable tool for physiology and pathology researchers, while also providing essential guidance for otorhinolaryngologists and endocrinologists in the diagnosis of Pendred syndrome and pendrin-related deafness.




Epithelial Anion Transport in Health and Disease


Book Description

Cl- absorption and HCO3- secretion are intimately associated processes vital to epithelial function, itself a key physiological activity. Until recently the transporters responsible remained obscure, but a breakthrough occurred with the discovery of the SLC26 transporters family. It is now clear that the SLC26 transporters have broad physiological functions since mutations in several members are linked to a variety of diseases. This book describes the properties of this family in detail, with contributions from the leading global researchers in the field. Complementary views from experts on other ion channels are offered in the discussions, which make fascinating reading. This family consists of at least 10 genes, each of which has several splice variants. Most members of the family are expressed in the luminal membrane of epithelial cells. Characterization of anion transport by three members has revealed that all function as Cl-/HCO3- exchangers, suggesting that SLC26 transporters are responsible for the luminal Cl-/HCO3- exchange activity. The SLC26 transporters are activated by the CF transmembrane conductance regulator and activate it in turn, leading to a model in which these molecules act together to mediate epithelial Cl- absorption and HCO3- secretion. The book includes chapters on the transport of other molecules by the SLC26 family, including oxalate in the kidney and sugars in cochlear hair cells amongst others. It also describes recent discoveries that most SLC26 transporters bind to scaffold proteins and that they all contain a conserved domain predicted to participate in protein-protein interactions. These suggest the SLC26 transporters exist in complexes with other Cl- and HCO3- transporters, and possibly other regulatory proteins. This book explores the functional role of these interactions, leading to better understanding of transepithelial fluid and electrolyte secretion and the diseases associated with it.




Ion Channels and Transporters of Epithelia in Health and Disease


Book Description

This book sheds new light on the physiology, molecular biology and pathophysiology of epithelial ion channels and transporters. It combines the basic cellular models and functions by means of a compelling clinical perspective, addressing aspects from the laboratory bench to the bedside. The individual chapters, written by leading scientists and clinicians, explore specific ion channels and transporters located in the epithelial tissues of the kidney, intestine, pancreas and respiratory tract, all of which play a crucial part in maintaining homeostasis. Further topics include the fundamentals of epithelial transport; mathematical modeling of ion transport; cell volume regulation; membrane protein folding and trafficking; transepithelial transport functions; and lastly, a discussion of transport proteins as potential pharmacological targets with a focus on the pharmacology of potassium channels.




Carbonates—Advances in Research and Application: 2012 Edition


Book Description

Carbonates—Advances in Research and Application: 2012 Edition is a ScholarlyEditions™ eBook that delivers timely, authoritative, and comprehensive information about Carbonates. The editors have built Carbonates—Advances in Research and Application: 2012 Edition on the vast information databases of ScholarlyNews.™ You can expect the information about Carbonates in this eBook to be deeper than what you can access anywhere else, as well as consistently reliable, authoritative, informed, and relevant. The content of Carbonates—Advances in Research and Application: 2012 Edition has been produced by the world’s leading scientists, engineers, analysts, research institutions, and companies. All of the content is from peer-reviewed sources, and all of it is written, assembled, and edited by the editors at ScholarlyEditions™ and available exclusively from us. You now have a source you can cite with authority, confidence, and credibility. More information is available at http://www.ScholarlyEditions.com/.




Chronic Obstructive Pulmonary Disease: New Insights for the Healthcare Professional: 2012 Edition


Book Description

Chronic Obstructive Pulmonary Disease: New Insights for the Healthcare Professional / 2012 Edition is a ScholarlyBrief™ that delivers timely, authoritative, comprehensive, and specialized information about Chronic Obstructive Pulmonary Disease in a concise format. The editors have built Chronic Obstructive Pulmonary Disease: New Insights for the Healthcare Professional / 2012 Edition on the vast information databases of ScholarlyNews.™ You can expect the information about Chronic Obstructive Pulmonary Disease in this eBook to be deeper than what you can access anywhere else, as well as consistently reliable, authoritative, informed, and relevant. The content of Chronic Obstructive Pulmonary Disease: New Insights for the Healthcare Professional / 2012 Edition has been produced by the world’s leading scientists, engineers, analysts, research institutions, and companies. All of the content is from peer-reviewed sources, and all of it is written, assembled, and edited by the editors at ScholarlyEditions™ and available exclusively from us. You now have a source you can cite with authority, confidence, and credibility. More information is available at http://www.ScholarlyEditions.com/.




Physiology and Pathology of Chloride Transporters and Channels in the Nervous System


Book Description

The importance of chloride ions in cell physiology has not been fully recognized until recently, in spite of the fact that chloride (Cl-), together with bicarbonate, is the most abundant free anion in animal cells, and performs or determines fundamental biological functions in all tissues. For many years it was thought that Cl- was distributed in thermodynamic equilibrium across the plasma membrane of most cells. Research carried out during the last couple of decades has led to a dramatic change in this simplistic view. We now know that most animal cells, neurons included, exhibit a non-equilibrium distribution of Cl- across their plasma membranes. Over the last 10 to 15 years, with the growth of molecular biology and the advent of new optical methods, an enormous amount of exciting new information has become available on the molecular structure and function of Cl- channels and carriers. In nerve cells, Cl- channels and carriers play key functional roles in GABA- and glycine-mediated synaptic inhibition, neuronal growth and development, extracellular potassium scavenging, sensory-transduction, neurotransmitter uptake and cell volume control. Disruption of Cl- homeostasis in neurons underlies pathological conditions such as epilepsy, deafness, imbalance, brain edema and ischemia, pain and neurogenic inflammation. This book is about how chloride ions are regulated and how they cross the plasma membrane of neurons. It spans from molecular structure and function of carriers and channels involved in Cl- transport to their role in various diseases. The first comprehensive book on the structure, molecular biology, cell physiology, and role in diseases of chloride transporters / channels in the nervous system in almost 20 years Chloride is the most abundant free anion in animal cells. THis book summarizes and integrates for the first time the important research of the past two decades that has shown that Cl- channels and carriers play key functional roles in GABA- and glycine-mediated synaptic inhibition, neuronal growth and development, extracellular potassium scavenging, sensory-transduction, neurotransmitter uptake and cell volume control The first book that systematically discusses the result of disruption of Cl- homeostasis in neurons which underlies pathological conditions such as epilepsy, deafness, imbalance, brain edema and ischemia, pain and neurogenic inflammation Spanning topics from molecular structure and function of carriers and channels involved in Cl- transport to their role in various diseases Involves all of the leading researchers in the field Includes an extensive introductory section that covers basic thermodynamic and kinetics aspects of Cl- transport, as well as current methods for studying Cl- regulation, spanning from fluorescent dyes in single cells to knock-out models to make the book available for a growing population of graduate students and postdocs entering the field




Basic Epithelial Ion Transport Principles and Function


Book Description

This book discusses unique ion channels and transporters that are located within epithelial tissues of various organs including the kidney, intestine, pancreas and respiratory tract. As the authors show, these channels and transporters play crucial roles in transepithelial ion and fluid transport across epithelia and their contribution to maintaining homeostasis. Readers will be introduced to the fundamentals of ion transport in terms of function, modelling, regulation, structure and pharmacology. This is the first of three volumes highlighting the importance of epithelial ion channels and transporters in basic physiology and pathophysiology of human diseases. This volume focuses on basic fundamentals of epithelial transport physiology. There is a range of chapters dedicated to specific aspects of epithelial ion transport and cell function. Accordingly, the authors discuss techniques used to determine epithelial function, principles of epithelia transport, polarization of epithelial cells, mathematical modelling of epithelial ion transport, protein folding of ion channels, degradation epithelial ion channels, fundamentals of epithelial sodium, potassium and chloride transport, fundamentals of bicarbonate secretion, volume regulation, and microRNA regulation of epithelial channels and transporters. Given its scope, Volume 1 offers a valuable resource for physiology students, scientists and clinicians alike.




Seldin and Giebisch's The Kidney


Book Description

A classic nephrology reference for over 25years, Seldin and Giebisch’s The Kidney, is the acknowledged authority on renal physiology and pathophysiology. In this 5th edition, such new and powerful disciplines as genetics and cell biology have been deployed to deepen and widen further the explanatory framework. Not only have previous chapters been extensively updated, but new chapters have been added to incorporate additional disciplines. Individual chapters, for example, now provide detailed treatment of the significance of cilia; the role of stem cells is now given special consideration. Finally, there has been a significant expansion of the section of pathophysiology, incorporating the newer findings of cell biology and genetics. If you research the development of normal renal function or the mechanisms underlying renal disease, Seldin and Giebisch’s The Kidney is your number one source for information. Offers the most comprehensive coverage on the market of fluid and electrolyte regulation and dysregulation in 85 completely revised chapters and 10 new chapters Includes 4sections, 62 chapters, devoted to regulation and disorders of acid-base homeostasis, and epithelial and nonepithelial transport regulation Includes foreword by Donald Seldin and Gerhard Giebisch, world renowned names in nephrology and editors of the previous three editions




Calcium Entry Channels in Non-Excitable Cells


Book Description

Calcium Entry Channels in Non-Excitable Cells focuses on methods of investigating the structure and function of non-voltage gated calcium channels. Each chapter presents important discoveries in calcium entry pathways, specifically dealing with the molecular identification of store-operated calcium channels which were reviewed by earlier volumes in the Methods in Signal Transduction series. Crystallographic and pharmacological approaches to the study of calcium channels of epithelial cells are also discussed. Calcium ion is a messenger in most cell types. Whereas voltage gated calcium channels have been studied extensively, the non-voltage gated calcium entry channel genes have only been identified relatively recently. The book will fill this important niche.