Titlebook: Agammaglobulinemia; Alessandro Plebani,Vassilios Lougaris Book 2015 Springer International Publishing Switzerland 2015 Autosomal recessive

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Early B Cell Development,cription factor activities, B cell precursors pass through defined stages of development. RAG-dependent rearrangement of V, D, and J heavy-chain gene segments starts in the pro-B cell stage. After the successful rearrangement of immunoglobulin gene segments, B cell precursors express functional immu

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Pulmonary Pathology in Agammaglobulinemia: Diagnosis and Treatment,oduction, and impaired airway clearance leads to structural airway damage, in particular to bronchiectasis. This chapter discusses the diagnostic and therapeutic approach to the patient with agammaglobulinemia with lung disease. The discussion refers to the available evidence with XLA patients and t

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,Bruton’s Tyrosine Kinase (BTK) Beyond B Lymphocytes: A Protein Kinase with Relevance in Innate Immufor early B cell development in the bone marrow and for B cell activation and differentiation in peripheral lymphoid organs. In addition to its role in B cell development and function, BTK is also expressed in myeloid cell populations. Several groups have investigated the function of BTK in non-B ce

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