Regulation Of Tissue Responses The Tweak Fn14 Pathway And Other Tnf Tnfr Superfamily Members That Activate Noncanonical Nfkb Signaling

The immune system mediates tissue responses under both physiological and pathological conditions, impacting the inflammatory, fibrogenic and regenerative components. In addition to various leukocyte subsets, it is now recognized that epithelial, endothelial and other non-hematopoietic tissue cell types actively contribute to the interplay shaping tissue responses. Further understanding the molecular pathways and mechanisms mediating these tissue responses is of great interest. In the past decade, TNF-like weak inducer of apoptosis (TWEAK) and its receptor, FGF-inducible molecule-14 (Fn14), members of the TNF/TNFR superfamily, have emerged as a prominent molecular axis regulating tissue responses. Generally leukocyte-derived, TWEAK signals through Fn14 which is highly induced in injured and diseased tissues on the surface of parenchymal, stromal and progenitor cells, thereby orchestrating a host of tissue-shaping responses, including inflammation, angiogenesis, cell proliferation or death, and the regulation of progenitor cells. Compelling preclinical results indicate that whereas transient TWEAK/Fn14 activation promotes productive tissue responses after acute injury, excessive or persistent TWEAK/Fn14 activation drives pathological tissue responses, leading to progressive damage and degeneration in target organs of injury, autoimmune and inflammatory diseases and cancer. Given that the highly inducible pattern of Fn14 expression is well conserved between mouse and man, the role of TWEAK/Fn14 in human disease is an area of intense investigation. Recent findings have also begun to shed light on how the TWEAK/Fn14 pathway fits into the immune network, interplaying with other well-established pathways, including TNFa, IL-17, IL-13 and TGFb, in regulating tissue responses. The noncanonical nuclear factor kB (NF?B) pathway plays a role in immunity and disease pathologies and appears to be activated by only a subset of TNF/ TNFR superfamily members. Of the various signaling pathways downstream of TWEAK/Fn14, particular attention has been placed on the noncanonical NF?B pathway given that given that TWEAK induces acute activation of canonical NF?B but prolonged activation of noncanonical pathway. Thus dovetailing of the TWEAK/Fn14 axis with noncanonical NF?B pathway activation may be a key mechanism underlying tissue responses. Also of great interest is a deeper understanding of where, when and how tissue responses are regulated by other TNF/ TNFR superfamily members that can signal through noncanonical NF?B. This Research Topic issue will cover: 1. TWEAK/Fn14 pathway biology, role in tissue responses, injury, and disease pathogenesis 2. Role of noncanonical NFkB signaling cascade in tissue responses 3. Translational studies of relevance of TWEAK/Fn14 and noncanonical NFkB in human disease 4. Other TNF superfamily members’ signaling through noncanonical NFkB in the regulation of tissue responses 5. Reviews and Perspectives on the above

The number of diagnosed cases of primary immunodeficiency diseases (PIDs) – a group of inborn disorders of the immune system – is growing rapidly, but misdiagnosis or late diagnosis still occurs in a significant number of patients, with serious consequences. This is the second edition of a practical reference textbook on PIDs that has been widely welcomed by scientists and clinicians from around the world. The new edition has been extensively revised to reflect advances in knowledge and includes various PIDs not previously covered. For each disease, information is provided on definition, etiology, clinical manifestations, diagnosis, and management. This book will represent an ideal resource for specialists when engaging in diagnosis, clinical decision-making, and treatment planning. It will also prove invaluable for doctors in training and other physicians and nurses who wish to learn more about PIDs.

Infections in Systemic Autoimmune Diseases: Risk Factors and Management, Volume Sixteen describes the state-of-the-art of the risk factors and management treating the most common systemic autoimmune diseases (SADS). This updated volume consists of an introductory chapter that provides a brief overview of what different types of infectious diseases exist, followed by eight chapters detailing risk factors, guidelines and recommendations per different disease and bacterial infections. International in scope, the list of more than 20 contributors from Europa and America reads like a who's who of clinical researchers in the field. Written by leading international clinical/surgical and scientific experts on Infection problems in systemic autoimmune diseases Provides a practical guide to the safer treatment of infections in patients with autoimmune diseases Includes a number of problems and recommendation that cannot be found in other books Designed as a 'guide to clinical practice' for management and risk interventions required by patients with SAD's

This volume provides the current understanding of death receptor's/TLR3 signaling regulation in cancer. Death receptors, including TRAIL-R1, TRAIL-R2, Fas and TNF-RI, owing to their ability to trigger apoptosis and to contribute to the elimination of cancer cells by the immune system have been considered, to variable extent, as important therapeutic targets for cancer therapy. But an increasing body of evidence suggests that some of these receptors may also contribute to tumorigenesis, or that new players such as TLR3 may be targeted for cancer therapy due to their ability to behave like death receptors.

Encyclopedia of Bone Biology, Three Volume Set covers hot topics from within the rapidly expanding field of bone biology and skeletal research, enabling a complete understanding of both bone physiology and its relation to other organs and pathophysiology. This encyclopedia will serve as a vital resource for those involved in bone research, research in other fields that cross link with bone, such as metabolism and immunology, and physicians who treat bone diseases. Each article provides a comprehensive overview of the selected topic to inform a broad spectrum of readers from advanced undergraduate students to research professionals. Chapters also explore the latest advances and hot topics that have emerged in recent years, including the Hematopoietic Niche and Nuclear Receptors. In the electronic edition, each chapter will include hyperlinked references and further readings as well as cross-references to related articles. Incorporates perspectives from experts working within the domains of biomedicine, including physiology, pathobiology, pharmacology, immunology, endocrinology, orthopedics and metabolism Provides an authoritative introduction for non-specialists and readers from undergraduate level upwards, as well as up-to-date foundational content for those familiar with the field Includes multimedia features, cross-references and color images/videos

Stiehm’s Immune Deficiencies: Inborn Errors in Immunity, Second Edition, is ideal for physicians and other caregivers who specialize in immunology, allergies, infectious diseases and pulmonary medicine. It provides a validated source of information for care delivery to patients, covering approaches to diagnosis that use both new genetic information and emphasize screening strategies. Management has changed dramatically over the past five years, so approaches to infection and autoimmunity are emphasized in an effort to improve outcomes and disseminate new information on the uses of targeted therapy. Covers immune deficiencies that are presented in a practical way, providing helpful information for active clinicians Fills an increasingly deep gap in the information available to clinicians Presents both clinical management and scientific advances for immune deficiencies Provides a primary resource for physicians in the field of immunodeficiencies Includes website access to a range of videos relevant to the topics discussed

Amphioxus Immunity: Tracing the Origin of Human Immunity covers a remarkable range of information about Amphioxus and its evolutionary context. This compilation of what is currently known about Amphioxus, with a sharp focus on its immune system, includes 13 topics, such as: Amphioxus as a model for understanding the evolution of vertebrates basic knowledge of immunology immune organs and cells of amphioxus a genomic and transcriptomic view of the Amphioxus immunity pattern recognition system in Amphioxus transcription factors in Amphioxus the complement system of Amphioxus the oxidative burst system in Amphioxus immune effectors in Amphioxus lipid signaling of immune response in Amphioxus apoptosis in amphioxus; primitive adaptive immune system of Amphioxus and future research directions This valuable reference book is loaded with information that will be useful for anyone who wishes to learn more about the origin of vertebrates and adaptive immunity. Provides new evidence on the origin of the adaptive immune system, the evolution of innate immunity, and evolution-stage specific immune defense mechanisms Not only presents the cells and molecules involved in the adaptive immune response in Amphioxus, but also characterizes the origination and evolution of the gene families and pathways involved in innate immunity Includes much pioneering work, from the molecular, genomic, and cellular to the individual level

Molecular Biology of B Cells, Second Edition is a comprehensive reference to how B cells are generated, selected, activated and engaged in antibody production. All of these developmental and stimulatory processes are described in molecular, immunological, and genetic terms to give a clear understanding of complex phenotypes. Molecular Biology of B Cells, Second Edition offers an integrated view of all aspects of B cells to produce a normal immune response as a constant, and the molecular basis of numerous diseases due to B cell abnormality. The new edition continues its success with updated research on microRNAs in B cell development and immunity, new developments in understanding lymphoma biology, and therapeutic targeting of B cells for clinical application. With updated research and continued comprehensive coverage of all aspects of B cell biology, Molecular Biology of B Cells, Second Edition is the definitive resource, vital for researchers across molecular biology, immunology and genetics.

This foundational work comprehensively examines the current state of the genetics, genomics and brain circuitry of psychiatric and neurological disorders. It consolidates discoveries of specific genes and genomic regions associated with these conditions, the genetic and anatomic architecture of these syndromes, and addresses how recent advances in genomics are leading to a reappraisal of the biology underlying clinical neuroscience. In doing so, it critically examines the promise and limitations of these discoveries toward treatment, and to the interdisciplinary nature of understanding brain and behavior. Coverage includes new discoveries regarding autism, epilepsy, intellectual disability, dementias, movement disorders, language impairment, disorders of attention, schizophrenia, and bipolar disorder. Genomics, Circuits, and Pathways in Clinical Neuropsychiatry focuses on key concepts, challenges, findings, and methods in genetics, genomics, molecular pathways, brain circuitry, and related neurobiology of neurologic and psychiatric disorders. Provides interdisciplinary appeal in psychiatry, neurology, neuroscience, and genetics Identifies key concepts, methods, and findings Includes coverage of multiple disorders from autism to schizophrenia Reviews specific genes associated with disorders Discusses the genetic architecture of these syndromes Explains how recent findings are influencing the understanding of biology Clarifies the promise of these findings for future treatment

This volume explores some of the most exciting recent advances in basic research on animal models of molecular pathology. This series provides a forum for discussion of new discoveries, approaches, and ideas Contributions from leading scholars and industry experts Reference guide for researchers involved in molecular biology and related fields