Function And Dysfunction Of Complement Factor H

The Complement FactsBook contains entries on all components of the Complement System, including C1q and Lectins, C3 Family, Serine Proteases, Serum Regulators of Complement Activation, Cell Surface Proteins, and Terminal Pathway Proteins. Domain Structure diagrams are incorporated to clearly illustrate the relationships between all the complement proteins, both within families and between families. The FactsBook also includes the cDNA sequences, marked with intron/exon boundaries, which will facilitate genetic studies. Includes the cDNA sequences, marked with intron/exon boundaries, facilitating genetic studies Presents detailed structural information including cDNA and gene structure for all proteins Introduces complement function, simply described for each function Data is as up-to-date as possible, including unpublished work from many contributors Incorporates domain structures diagrams, which beautifully illustrate the relationship between all the complement proteins, both within, and between, familiesEach chapter has been written by an expert in the field Data is as up-to-date as possible, including unpublished work from many contributors Entries provide information on: Alternative nomenclature, Physiochemical properties, Structure and function, Tissue distribution and regulation expression, Protein sequence/modules, Chromosomal location, Genomic structure, Database accession numbers, Deficiency and polymorphic variants, Key references

Pediatric Allergy supplies the comprehensive guidance you need to diagnose, manage, and treat virtually any type of allergy seen in children. Drs. Leung, Sampson, Geha, and Szefler present the new full-color second edition, with coverage of the diagnosis and management of anaphylaxis, the immune mechanisms underlying allergic disease, the latest diagnostic tests, and more. Treat the full range of pediatric allergic and immunologic diseases through clinically focused coverage relevant to both allergists and pediatricians. Understand the care and treatment of pediatric patients thanks to clinical pearls discussing the best approaches. Easily refer to appendices that list common food allergies and autoantibodies in autoimmune diseases. Apply the newest diagnostic tests available—for asthma, upper respiratory allergy, and more—and know their benefits and contraindications. Treat the allergy at its source rather than the resulting reactions through an understanding of the immune mechanisms underlying allergic diseases. Get coverage of new research that affects methods of patient treatment and discusses potential reasons for increased allergies in some individuals. Better manage potential anaphylaxis cases through analysis of contributing facts and progression of allergic disease. Effectively control asthma and monitor its progression using the new step-by-step approach. Eliminate difficulty in prescribing antibiotics thanks to coverage of drug allergies and cross-reactivity.

This book provides a comprehensive account of the pathogenesis, clinical features, and management of ocular vascular occlusive disorders, with the focus very much on the scientific evidence. This offers a sound basis for addressing the many controversies that surround these disorders, which collectively constitute the most common cause of visual impairment or blindness. The book is divided into two sections, the first of which addresses the basic science and encompasses vascular anatomy, blood supply and flow, and retinal tolerance time to acute ischemia. The second, clinical, section covers the presentation, clinical features, diagnosis, and treatment of the full range of vascular occlusive disorders of the retina, the choroid, the anterior segment of the eye, ophthalmic manifestations of carotid artery disease and the optic nerve. Ocular Vascular Occlusive Disorders, written by a distinguished world leader in the field, will be invaluable for general ophthalmologists, and particularly for retina specialists, neuro-ophthalmologists, and researchers.

Offering a broad appeal to microbiologists, immunologists, and infectious disease specialists, this four volume encyclopedia covers all autoimmune, tropical, and infectious diseases. Emphasis will also be placed on genetics, physiology, metabolism, pathogenesis and applied microbiology. Under the leadership of some of the most world renowned names in the field, the encyclopedia will bring together an outstanding collection of contributions by top scientists in a variety of fields. Volumes 1-3: Diseases will be divided by the 11 main sections of the body, namely Integumentary, Skeletal, Respiratory, Digestive, Urinary, and Reproductive. For some of the autoimmune disease, more then one system will be involved but the delineation serves to broadly break down the diseases into systems. Volume 4 will cover the vaccines for said diseases and future prospects will be offered by leaders in industry and academia. Volume 4 will also be broken down into all the body systems, as in the other two volumes. For each vaccine, for each disease, and in each system the following will be included: • A list of the vaccines currently available along with a list of the companies that manufacture them • Molecular Immunology of the Vaccine • Type of Immunity involved in protection • Mode of Vaccination for each vaccine; repeated boosters and length of immunological memory • Commercial production of vaccines • Storage of vaccines • Standardization and Control of Vaccines • WHO programs and World-Wide Disease Eradication Programs based upon Vaccines.

From small beginnings in the early 1970s, the study of complement regulatory proteins has grown in the last decade to the point where it dominates the complement field. This growth has been fueled by the discovery of new regulators, the cloning of old and new regulators, the discovery that many of the regulators are structurally and evolutionarily related to each other and the development of recombinant forms for use in therapy. There are now more proteins known to be involved in controlling the complement system than there are components of the system and the list continues to grow. The time is ripe for a comprehensive review of our current knowledge of these intriguing proteins. This book does just that. The first few chapters discuss the "nuts-and-bolts" of the complement regulators, describing their structures, functional roles and modes of action. The roles of the complement regulators in vivo are then described, focusing on the consequences of deficiency, roles in the reproductive system, interactions with pathogens and exploitation for therapy. The interesting developments in defining the complement regulators expressed in other species are also discussed. The book is written as a monograph, albeit by two people. The text is as readable as possible without compromising on scientific accuracy and completeness. The conversational style very evident in some sections is deliberate! Placing all references in a single bibliography at the end of the text further improves readability. The reader will go to the book to discover a specific fact but be persuaded to read more and derive pleasure from the process. The authors' enthusiasm for the subject comes over strongly in the text, and this enthusiasm proves infectious. Complement regulators--structure, functional roles and mode of action Comprehensive reviews of each of the individual regulators Roles of Complement regulators in vivo,in health and disease: Consequences of deficiency Roles in the reproductive system Interactions with pathogens Exploitation for therapy Complement regulators in other species

The complement system is an important innate immune surveillance network that has a key role in protecting our bodies against pathogens and other threats. It is a highly complex system consisting of approximately 50 soluble and cell surface-bound proteins that interact to eliminate danger signals. These signals include factors such as invading microorganisms, necrotic cells, and immune complexes. Furthermore, complement can link innate and adaptive immune responses by regulating T cell and B cell responses. The complement system is tightly regulated to avoid uncontrolled activation. Dysregulation of the complement system has been linked to numerous diseases, both rare and common. Uncontrolled or exaggerated activation can lead to life-threatening conditions such as dysregulation of coagulation, fibrinolysis, systemic inflammation and shock, and possible failure of a variety of organs such as the eyes, kidneys, skin, brain, and vascular system. In recent years, the complement system has gained increased interest, especially as a promising candidate for therapeutic intervention. Eculizumab, the first approved complement inhibitor, is highly effective for treating atypical hemolytic uremic syndrome (aHUS) and paroxysmal nocturnal hemoglobinuria (PNH). Many more drug candidates are currently evaluated in ongoing clinical trials.

This reference presents detailed discussions of the history, pathology, pathophysiology, and approaches to treatment of the complicated, constantly evolving syndromes known as thrombotic thromocytopenic purpura (TTP), from many different points of view. Hemolytic Uremic syndrome and Thrombotic Thrombocytopenic Purpura offers: extensive analyses of the relationship between HUS and TTP; epidemiological studies of HUS from the UK, Canada, Asia, South Africa and Argentina; investigations of non-renal complications of HUS; perspectives on atypical HUS and post-transplantation HUS; delineations of the association between verotoxin and HUS, HUS and pregnancy, and HUS and cancer and cancer tharapy; information on HUS/TTP in HIV-infected patients; explications of the pathology and pathogenesis of HUS; and approaches to treatment of HUS, prognosis, and long-term follow-up.;In addition, it covers the history and pathogenesis of TTP, von Willebrand factor abnormalities in TTP and HUS, platelet agglutinating proteins in TTP, and the treatment of TTP.;With over 2000 literature citations and figures, this book is for nephrologists, hematologists, oncologists, paediatricians, pathologists, gastroenterologists, internists, endocrinologists, infectious disease specialists, neurologists, gynaecologists, microbiologists, surgeons, geneticsts, epidemiologists, radiologists, and medical school students in these disciplines.