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  1. Book ; Online ; E-Book: Primary immunodeficiency diseases

    Rezaei, Nima / Aghamohammadi, Asghar / Notarangelo, Luigi D.

    definition, diagnosis, and management

    2017  

    Author's details Nima Rezaei, Asghar Aghamohammadi, Luigi D. Notarangelo editors
    Keywords Agammaglobulinemia ; Antibody Deficiencies ; Autoimmunity ; Autoinflammatory Disorders ; B-cell immunodeficiency ; Combined Immunodeficiencies ; Dendritic Cell Deficiencies ; Genetic Defects ; Immunology ; Infectious Complications ; Monocyte Deficiencies ; Phagocyte Defects ; T-Cell Immunodeficiency ; Treatment
    Language English
    Size 1 Online-Ressource (xvii, 582 Seiten), Illustrationen
    Edition Second edition
    Publisher Springer
    Publishing place Berlin
    Publishing country Germany
    Document type Book ; Online ; E-Book
    Remark Zugriff für angemeldete ZB MED-Nutzerinnen und -Nutzer
    HBZ-ID HT019283766
    ISBN 978-3-662-52909-6 ; 9783662529072 ; 3-662-52909-2 ; 3662529076
    DOI 10.1007/978-3-662-52909-6
    Database ZB MED Catalogue: Medicine, Health, Nutrition, Environment, Agriculture

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  2. Article ; Online: Rubella Virus-Associated Granulomas in Immunocompetent Adults-Possible Implications.

    Notarangelo, Luigi D

    JAMA dermatology

    2022  Volume 158, Issue 6, Page(s) 611–613

    MeSH term(s) Adult ; Granuloma/diagnosis ; Granuloma/etiology ; Humans ; Rubella/complications ; Rubella/diagnosis ; Rubella virus
    Language English
    Publishing date 2022-03-25
    Publishing country United States
    Document type Journal Article ; Comment
    ZDB-ID 2701761-8
    ISSN 2168-6084 ; 2168-6068
    ISSN (online) 2168-6084
    ISSN 2168-6068
    DOI 10.1001/jamadermatol.2022.0055
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Ui VI Zs.32: Show issues Location:
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    ab Jg. 2022: Lesesaal (EG)

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  3. Book ; Online: NK Cell Subsets in Health and Disease: New Developments

    Orange, Jordan S. / Notarangelo, Luigi D. / Marcenaro, Emanuela / Vivier, Eric

    2017  

    Abstract: Natural Killer (NK) cells were discovered ca 1975, as the first group of lymphoid cells that were neither T cells nor B cells. Since then, the dissection of the biology of NK cells has been growing exponentially with many seminal discoveries from the ... ...

    Abstract Natural Killer (NK) cells were discovered ca 1975, as the first group of lymphoid cells that were neither T cells nor B cells. Since then, the dissection of the biology of NK cells has been growing exponentially with many seminal discoveries from the identification of MHC class I-specific inhibitory receptors to the discovery of receptor-ligand pairs involved in NK cell activation and to the manipulation of NK cells in cancer. In this research topic, we asked a group of thought leaders in NK cell biology to review recent advances in their origins and biology, and their roles in cancer, infection and inflammation. Together, these 25 articles provide a timely survey of NK cells as critical immunologic components of health and disease. They will hopefully prompt further dialogue and developments in basic and translational immunology
    Keywords Medicine (General) ; Immunologic diseases. Allergy
    Size 1 electronic resource (243 p.)
    Publisher Frontiers Media SA
    Document type Book ; Online
    Note English ; Open Access
    HBZ-ID HT020097480
    ISBN 9782889453504 ; 2889453502
    Database ZB MED Catalogue: Medicine, Health, Nutrition, Environment, Agriculture

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  4. Article ; Online: Human thymus in health and disease: Recent advances in diagnosis and biology.

    Bosticardo, Marita / Notarangelo, Luigi D

    Seminars in immunology

    2023  Volume 66, Page(s) 101732

    Abstract: The thymus is the crucial tissue where thymocytes develop from hematopoietic precursors that originate from the bone marrow and differentiate to generate a repertoire of mature T cells able to respond to foreign antigens while remaining tolerant to self- ... ...

    Abstract The thymus is the crucial tissue where thymocytes develop from hematopoietic precursors that originate from the bone marrow and differentiate to generate a repertoire of mature T cells able to respond to foreign antigens while remaining tolerant to self-antigens. Until recently, most of the knowledge on thymus biology and its cellular and molecular complexity have been obtained through studies in animal models, because of the difficulty to gain access to thymic tissue in humans and the lack of in vitro models able to faithfully recapitulate the thymic microenvironment. This review focuses on recent advances in the understanding of human thymus biology in health and disease obtained through the use of innovative experimental techniques (eg. single cell RNA sequencing, scRNAseq), diagnostic tools (eg. next generation sequencing), and in vitro models of T-cell differentiation (artificial thymic organoids) and thymus development (eg. thymic epithelial cell differentiation from embryonic stem cells or induced pluripotent stem cells).
    MeSH term(s) Animals ; Humans ; T-Lymphocytes ; Thymus Gland ; Cell Differentiation ; Epithelial Cells ; Biology
    Language English
    Publishing date 2023-02-28
    Publishing country England
    Document type Journal Article ; Review ; Research Support, N.I.H., Intramural
    ZDB-ID 1018141-6
    ISSN 1096-3618 ; 1044-5323
    ISSN (online) 1096-3618
    ISSN 1044-5323
    DOI 10.1016/j.smim.2023.101732
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 2843: Show issues Location:
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  5. Article ; Online: Gene therapy for inborn errors of immunity: Base editing comes into play.

    Malech, Harry L / Notarangelo, Luigi D

    Cell

    2023  Volume 186, Issue 7, Page(s) 1302–1304

    Abstract: CRISPR-Cas9-based base editing allows precise base editing to achieve conversion of adenosine to guanine or cytosine to thymidine. In this issue of Cell, McAuley et al. use adenine base editing to correct a single base-pair mutation causing human CD3δ ... ...

    Abstract CRISPR-Cas9-based base editing allows precise base editing to achieve conversion of adenosine to guanine or cytosine to thymidine. In this issue of Cell, McAuley et al. use adenine base editing to correct a single base-pair mutation causing human CD3δ deficiency, demonstrating superior efficiency of genetic correction with reduced undesired genetic alterations compared with standard CRISPR-Cas9 editing.
    MeSH term(s) Humans ; Adenine ; CRISPR-Cas Systems/genetics ; Gene Editing ; Genetic Therapy ; Mutation ; Genetic Diseases, Inborn/genetics ; Genetic Diseases, Inborn/therapy ; Immune System Diseases/genetics ; Immune System Diseases/therapy
    Chemical Substances Adenine (JAC85A2161)
    Language English
    Publishing date 2023-03-31
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Intramural ; Comment
    ZDB-ID 187009-9
    ISSN 1097-4172 ; 0092-8674
    ISSN (online) 1097-4172
    ISSN 0092-8674
    DOI 10.1016/j.cell.2023.03.001
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 1167: Show issues Location:
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    Zs.MG 58: Show issues

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  6. Book: The year in human and medical genetics / 3 / iss. ed. Jean-Laurent Casanova ; Mary Ellen Conley and Luigi Notarangelo

    Casanova, Jean-Laurent / Conley, Mary Ellen / Notarangelo, Luigi D.

    inborn errors of immunity

    (Annals of the New York Academy of Science ; 1250)

    2012  

    Series title Annals of the New York Academy of Science ; 1250
    Annals of the New York Academy of Sciences
    The year in human and medical genetics
    Collection Annals of the New York Academy of Sciences
    The year in human and medical genetics
    Language English
    Size 79 S. : Ill., graph. Darst.
    Publisher Blackwell
    Publishing place Boston, Mass
    Publishing country United States
    Document type Book
    HBZ-ID HT017192386
    ISBN 1-57331-878-7 ; 978-1-57331-878-5
    Database Catalogue ZB MED Medicine, Health

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    In stock of ZB MED Cologne/Königswinter

    Shelf markLoan statusLocation
    Se 110 -1250- verfügbar in Königswinter Königswinter

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  7. Book: The year in human and medical genetics / 2 / iss. ed. Jean-Laurent Casanova ...

    Casanova, Jean-Laurent / Conley, Mary Ellen / Notarangelo, Luigi D.

    inborn errors of immunity

    (Annals of the New York Academy of Science ; 1246)

    2011  

    Series title Annals of the New York Academy of Science ; 1246
    The year in human and medical genetics
    Annals of the New York Academy of Sciences
    Collection The year in human and medical genetics
    Annals of the New York Academy of Sciences
    Language English
    Size 141 S. : Ill., graph. Darst.
    Publisher Blackwell
    Publishing place Boston, Mass
    Publishing country United States
    Document type Book
    HBZ-ID HT017125179
    ISBN 1-57331-851-5 ; 978-1-57331-851-8
    Database Catalogue ZB MED Medicine, Health

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    In stock of ZB MED Cologne/Königswinter

    Shelf markLoan statusLocation
    Se 110 -1246- verfügbar in Königswinter Königswinter

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  8. Book: The year in human and medical genetics / 1 / iss. ed. Jean-Laurent Casanova ; Mary Ellen Conley and Luigi Notarangelo

    Casanova, Jean-Laurent / Conley, Mary Ellen / Notarangelo, Luigi D.

    inborn errors of immunity

    (Annals of the New York Academy of Science ; 1238)

    2011  

    Series title Annals of the New York Academy of Science ; 1238
    Annals of the New York Academy of Sciences
    The year in human and medical genetics
    Collection Annals of the New York Academy of Sciences
    The year in human and medical genetics
    Language English
    Size 144 S. : Ill., graph. Darst.
    Publisher Blackwell
    Publishing place Boston, Mass
    Publishing country United States
    Document type Book
    HBZ-ID HT017090462
    ISBN 1-57331-849-3 ; 978-1-57331-849-5
    Database Catalogue ZB MED Medicine, Health

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    In stock of ZB MED Cologne/Königswinter

    Shelf markLoan statusLocation
    Se 110 -1238- verfügbar in Königswinter Königswinter

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  9. Book ; Collection: The year in human and medical genetics

    Casanova, Jean-Laurent / Conley, Mary Ellen / Notarangelo, Luigi D.

    inborn errors of immunity

    (Annals of the New York Academy of Sciences ; ...)

    2011  

    Series title Annals of the New York Academy of Sciences
    ...
    Language English
    Dates of publication 2011-9999
    Publisher Blackwell
    Publishing place Boston, Mass
    Publishing country United States
    Document type Book ; Collection (display volumes)
    HBZ-ID HT017090431
    Database Catalogue ZB MED Medicine, Health

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  10. Article ; Online: Interferons in Down syndrome: When more is less.

    Notarangelo, Luigi D / Bosticardo, Marita

    Immunity

    2022  Volume 55, Issue 11, Page(s) 1967–1969

    Abstract: Individuals with Down syndrome (DS) are at a lower risk for viral infections than the general population, yet their infectious episodes are often more serious. In this issue of Immunity, Malle et al. provide important mechanistic insight into this ... ...

    Abstract Individuals with Down syndrome (DS) are at a lower risk for viral infections than the general population, yet their infectious episodes are often more serious. In this issue of Immunity, Malle et al. provide important mechanistic insight into this paradox, showing that individuals with DS have dysregulated IFN-I responses with increased initial signaling translating into a refractory state that makes their immune systems less capable of controlling viral infections.
    MeSH term(s) Humans ; Interferons ; Down Syndrome ; Immunity, Innate ; Virus Diseases ; Signal Transduction
    Chemical Substances Interferons (9008-11-1)
    Language English
    Publishing date 2022-10-08
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Intramural ; Comment
    ZDB-ID 1217235-2
    ISSN 1097-4180 ; 1074-7613
    ISSN (online) 1097-4180
    ISSN 1074-7613
    DOI 10.1016/j.immuni.2022.10.011
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 4227: Show issues Location:
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    Zs.MG 69: Show issues

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