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Making Science Fun – A Tribute to Our Colleague and Friend, Prof. Antonius G. Rolink (1953–2017)
This Research Topic honors the memory of Prof. Antonius “Ton” G. Rolink (April 19, 1953–August 06, 2017), our colleague, mentor and friend in immunology. It is now over a year since Ton left us. This article collection, authored by many of Ton’s friends and colleagues, reflects the huge contribution to cellular and molecular immunology that work emanating directly from Ton’s own hands and laboratory have made to the understanding of lymphocyte development. Ton’s hard work, expertise, generosity, passion for science and infectious humor were legendary and for all of those lucky enough to have been his colleague, he ensured that science was fun. We take this opportunity of thanking...
Identifying Novel Inborn Errors of the Immune System
In her study Elisabeth Salzer describes three novel monogenic diseases. For CD27 deficiency Elisabeth Salzer describes a large cohort of patients. Although all patients shared the same causative missense mutation, they displayed diverse clinical presentations. In another patient she was able to identify a mutation in PRKCD resulting in a primary immunodeficiency with severe Lupus-like autoimmunity. The patient exhibited increased mRNA levels of IL6. Therefore, treatment with Tocilizumab, a humanized anti-IL-6 receptor monoclonal antibody was suggested. In a family with a history of deaths due to inflammatory bowel disease she identified a missense mutation in IL21. She produced wild type and mutated IL-21 protein and demonstrated a loss of function phenotype. As IL-21 is in clinical trials, she proposed a potentially curative treatment option. These discoveries contributed to the understanding of the multifaceted regulatory mechanisms of the immune system and highlighted essential players in these complex signaling networks.
Transgenesis and Targeted Mutagenesis in Immunology
Investigations into the field of immunology are rapidly expanding with the use of genetically altered mice at the embryonic stage. This breakthrough laboratory guide provides a complete study of transgenesis and targeted mutagenesis in laboratory mice that will be valued by researchers looking for fresh observations and interpretations when designing future experiments.Special Features Include:Contributions of two Nobel Prize winnersAddresses the use of mouse models in studying the immune systemTargets gene distribution in embryonic stem cells and their introduction into blastocyte mice modelsAnalyzes the in vivo functional loss of embryonic cellsA practical, useful guidebook for individual researchers, laboratories, and libraries
Advances in Mucosal Immunology
The Seventh International Congress of Mucosal Immunology held in Prague, the beautiful old capital of The Czech Republic, 16-20 August 1992, was the first to be sponsored by the Society for Mucosal Immunology, and was the largest since their inception 20 years earlier in Birmingham, Alabama. It was attended by 624 participants who gave 538 presentations, more than 10 times the numbers of the first meeting; these proceedings contain 354 papers that were submitted for publication. The political events in Europe that made it possible to hold this Congress in Prague also allowed for the first time the participation of large numbers of scientists from Eastern Europe, as weil as from Asia, and the...
Humoral Primary Immunodeficiencies
This book presents detailed state of the art knowledge on the humoral primary immunodeficiencies (PIDs), i.e., disorders arising from impaired antibody production due to defects intrinsic to B cells or defective interaction between B and T cells. There is extensive coverage of both basic science discoveries and the latest clinical advances in the field. The book is structured in accordance with the most recent classification of PIDs and also covers updates on the B cell immunological synapse. Readers will find comprehensive, in-depth descriptions of novel humoral PID genes and related clinical applications, mucosal B cells, and the various clinical phenotypes of humoral PIDs. Aspects such as differential diagnosis, clinical management in children and adults, and the role of vaccines are also addressed. The authors are all recognized experts from Europe, Australia, and the United States. Humoral Primary Immunodeficiencies will be of high value for immunologists, pediatricians, rheumatologists, oncologists, internists, and infectious disease specialists and will also be informative for MD and PhD students.
Agammaglobulinemia
This book provides an updated overview of agammaglobulinemia, a rare form of primary immunodeficiency which is considered the prototype of the congenital humoral defects, and which is characterized by the absence of peripheral B cells and very low serum immunoglobulin levels. The book opens by discussing the highly orchestrated early B cell development in the bone marrow and the genes involved based on both human and animal models. The pathogenesis and clinical presentation of X-linked agammaglobulinemia, caused by mutations in the BTK (Bruton’s tyrosine kinase) gene, are then presented in detail, followed by descriptions of the clinical manifestations and molecular basis of the less frequent autosomal recessive and autosomal dominant forms of agammaglobulinemia. Patients’ management in terms of respiratory complications, gammaglobulin replacement therapy and the potential value of novel experimental therapeutic strategies are discussed. The book’s closing chapters offer a comprehensive and updated description of mutations in the BTK gene, and the expression and function of BTK in cells other than B cells.
