Primary Cilia

In recent years, the role of cilia in the study of health, development and disease has been increasingly clear, and new discoveries have made this an exciting and important field of research. This comprehensive volume, a complement to the new three-volume treatment of cilia and flagella by King and Pazour, presents easy-to-follow protocols and detailed background information for researchers working with cilia and flagella. *Covers protocols for primary cilia across several systems and species * Both classic and state-of-the-art methods readily adaptable across model systems, and designed to last the test of time * Relevant to clinicians and scientists working in a wide range of fields

Cilia are tiny microtubule-based organelles projecting from the plasma membrane of practically all cells in the body. In the past 10 years a flurry of research has indicated a crucial role of this long-neglected organelle in the development and function of the central nervous system. A common theme of these studies is the critical dependency of signal transduction of the Sonic hedgehog, and more recently, Wnt signaling pathways upon cilia to regulate fate decisions and morphogenesis. Both primary and motile cilia also play crucial roles in the function of the nervous system, including the primary processing of sensory information, the control of body mass, and higher functions such as behavior and cognition, serving as "antennae" for neurons to sense and process their environment. In this book we describe the structure and function of cilia and the various tissues throughout the brain and spinal cord that are dependent upon cilia for their proper development and function.

The scope of the book is to highlight the diverse roles of cilia in human development and disease. Almost all cell types form cilia and although they were first detected about 200 years ago, their significance was unclear. In the past ten years, it has become obvious that cilia have got sensory functions, as well as roles in motility and their mis-formation or the deregulation of the signaling pathways they control has been associated with defective development and human disease. Although research has concentrated on the role of the cilium in each organ, no effort has been made so far to bring all this information together and relate it to the various human diseases. This book aims to gather all the expertise that has been acquired on primary cilia and translate it into a medical and research context that will be of interest to postgraduate students, researchers, medics and scientists.

Microcirculation is key to providing enough nutrition and oxygen from head to toe. This is possible only through an extensive network of blood vessels spread around the body. Effect of microcirculation abnormalities stretch beyond one’s comprehension. The effects could be felt at any age, from the foetal life to the adulthood. The chapters present in this book describe how these abnormalities could lead to diseases such as atherosclerosis, thrombosis, diabetes, hypertension. Disorders of microcirculation could be related to the structural and/or functional damage to the inner lining of the blood vessels. Early identification of these disorders could benefit many ailments including cardiovascular and cerebrovascular diseases such as heart attack and stroke.

This volume focuses on the etiology and morphogenesis of congenital heart diseases. It reviews in detail the early development and differentiation of the heart, and later morphologic events of the cardiovascular system, covering a wide range of topics such as gene functions, growth factors, transcription factors and cellular interactions that are implicated in cardiac morphogenesis and congenital heart disease. This book also presents recent advances in stem cell and cell sheet tissue engineering technologies which have the potential to provide novel in vitro disease models and to generate regenerative paradigms for cardiac repair and regeneration. This is the ideal resource for physician scientists and investigators looking for updates on recent investigations on the origins of congenital heart disease and potential future therapies.

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.

Cilia and Flagella presents protocols accessible to all individuals working with eukaryotic cilia and flagella. These recipes delineate laboratory methods and reagents, as well as critical steps and pitfalls of the procedures. The volume covers the roles of cilia and flagella in cell assembly and motility, the cell cycle, cell-cell recognition and other sensory functions, as well as human diseases and disorders. Students, researchers, professors, and clinicians should find the book's combination of "classic" and innovative techniques essential to the study of cilia and flagella. Key Features * A complete guide containing more than 80 concise technical chapters friendly to both the novice and experienced researcher * Covers protocols for cilia and flagella across systems and species from Chlamydomonas and Euglena to mammals * Both classic and state-of-the-art methods readily adaptable across model systems, and designed to last the test of time, including microscopy, electrophoresis, and PCR * Relevant to clinicians interested in respiratory disease, male infertility, and other syndromes, who need to learn biochemical, molecular, and genetic approaches to studying cilia, flagella, and related structures

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.