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Part I Overview; 1: C.I. Edvard Smith, Hans D. Ochs, and Jennifer M. Puck: Genetically Determined Immunodeficiency Diseases: A Perspective; 2: Jennifer M. Puck and Robert L. Nussbaum: Genetic Principles and Technologies in the Study of Immune Disorders; 3: Gerald J. Sprangrude: Mammalian Hematopoietic Development and Function; 4: Rae S. M. Yeung, Pamela S. Ohashi, Mary E. Saunders, and Tak W. Mak: T Cell Development; 5: Antonius Rolink, Jan Andersson, Ulf Grawunder, and Fritz Melchers: Molecular Mechanisms Guiding B Cell Development; 6: Neetu Gupta, Anthony L. DeFranco, and Arthur Weiss: Signal Transduction by T and B Lymphocyte Antigen Receptors; 7: Sirpa Jalkanen and Marko Salmi: Lymphoid Organ Development, Cell Trafficking, and Lymphocyte Responses; 8: Kuender D. Yang, Paul G. Quie, and Harry R. Hill: Phagocytic System; Part II Syndromes; 9: Jennifer M. Puck: X-linked Severe Combined Immunodeficiency; 10: Fabio Candotti and Luigi Notarangelo: Autosomal Recessive Severe Combined Immunodeficiency Due to Defects in Cytokine Signaling Pathways; 11: Jean-Pierre de Villartay, Klaus Schwarz, and Anna Villa: V(D)J Recombination Defects; 12: Rochelle Hirschhorn, and Fabio Candotti: Immunodeficiency Due to Defects of Purine Metabolism; 13: Tala A. Chatila and Markku Heikinheimo: Severe Combined Immunodeficiency Due to Mutations in the CD45 Gene; 14: Melissa E. Elder: Severe Combined Immunodeficiency Due to Defects in T Cell Receptor-Associated Protein Kinases; 15: Chaim M. Roifman: Human Interleukin-2 Receptor Alpha Deficiency; 16: Jose R. Regueiro and Teresa Espanol: CD3 and CD8 Deficiencies; 17: Walter Reith, Barbara Lisowska-Grospierre, and Alain Fischer: Molecular Basis of Major Histocompatibility Complex Class II Deficiency; 18: Henri de la Salle, Lionel Donato, and Daniel Hanau: Peptide Transporter Defects in Human Leukocyte Antigen Class I Deficiency; 19: Raif S. Geha, Alessandro Plebani, and Luigi D. Notarangelo: CD40, CD40 Ligand, and the Hyper-IgM Syndrome; 20: Anne Durandy, Patrick Revy, and Alain Fischer: Autosomal Hyper-IgM Syndromes Caused by an Intrinsic B Cell Defect; 21: C.I. Edvard Smith, Anne B. Satterthwaite, and Owen N. Witte: X-linked Agammaglobulinemia: A Disease of Btk Tyrosine Kinase; 22: Mary Ellen Conley: Autosomal Recessive Agammaglobulinemia; 23: Lennart Hammarstr and C.I. Edvard Smith: Genetic Approach to Common Variable Immunodeficiency and IgA Deficiency; 24: Jennifer M. Puck, Frederic Rieux-Laucat, Franise Le Deist, and Stephen E. Straus: Autoimmune Lymphoproliferative Syndrome; 25: Leena Peltonen-Palotie, Maria Halonen, and Jaakko Perheentupa: Autoimmune Polyendocrinopathy, Candidiasis, Ectodermal Dystrophy; 26: Troy R. Torgerson, Eleonora Gambineri, Steven F. Ziegler, and Hans D. Ochs : Immune Dysregulation, Polyendocrinopathy, Enteropathy, and X-Linked Inheritance; 27: Daniel L. Kastner, Susannah Brydges, and Keith M. Hull: Periodic Fever Syndromes; 28: Melanie J. Newport, Steven M. Holland, Michael Levin, and Jean-Laurent Casanova: Inherited Disorders of the Interleukin-12/23-Interferon Gamma Axis; 29: Martin F. Lavin and Yosef Shiloh: Ataxia-Telangiectasia; 30: Rolf-Dieter Wegner, James J. German, Krystyna H. Chrzanowska, Martin Digweed, and Markus Stumm: Chromosomal Instability Syndromes Other Than Ataxia-Telangiectasia; 31: Hans D. Ochs and Fred S. Rosen: Wiskott-Aldrich Syndrome; 32: Volker Schuster and Cox Terhorst: X-linked Lymphoproliferative Disease due to Defects of SH2D1A; 33: Deborah A. Driscoll and Kathleen E. Sullivan: DiGeorge Syndrome: A Chromosome 22q11.2 Deletion Syndrome; 34: Bodo Grimbacher, Jennifer M. Puck, and Steven M. Holland: Hyper-IgE Recurrent Infection Syndromes; 35: R. Scott Hansen, Corry Weemaes, and Cisca Wijmenga: Immunodeficiency with Centromere Instability and Facial Anomalies; 36: Mario Abinun, Ilkka Kaitila and Jean-Laurent Casanova: Immunodeficiencies with Associated Manifestations of Skin, Hair, Teeth, and Skeleton; 37: Dirk Roos, Taco W. Kuijpers, and John T. Curnutte: Chronic Granulomatous Disease; 38: Amos Etzioni and John M. Harlan: Cell Adhesion and Leukocyte Adhesion Defects; 39: David C. Dale and Andrew G. Aprikyan: Cyclic and Congenital Neutropenia Due to Defects in Neutrophil Elastase; 40: Richard A. Spritz: Chediak-Higashi Syndrome; 41: Genevie de Saint Basile: Inherited Hemophagocytic Syndromes; 42: Kathleen E. Sullivan and Jerry A. Winkelstein: Genetically Determined Deficiencies of the Complement System; Part III Assessment and Treatment; 43: Helen M. Chapel, Siraj Misbah, and David B. Webster: Assessment of the Immune System; 44: Jennifer M. Puck: Genetic Aspects of Primary Immunodeficiencies; 45: Jouni Viaho, Crina Samarghitean, Hilkka Piiril? Marianne Pusa, and Mauno Vihinen: Immunodeficiency Information Systems; 46: E. Richard Stiehm and Helen M. Chapel: Conventional Therapy of Primary Immunodeficiency Diseases; 47: Rebecca H. Buckley and Alain Fischer: Bone Marrow Transplantation for Primary Immunodeficiency Diseases; 48: Fabio Candotti and Alain Fischer: Gene Therapy

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