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  1. Article ; Online: Time for a new approach to drug development for rare systemic autoinflammatory diseases.

    Zitoun, Natalie M / Demirkaya, Erkan / Goldbach-Mansky, Raphaela / Romano, Micol

    Nature reviews. Rheumatology

    2024  

    Language English
    Publishing date 2024-04-02
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2491532-4
    ISSN 1759-4804 ; 1759-4790
    ISSN (online) 1759-4804
    ISSN 1759-4790
    DOI 10.1038/s41584-024-01108-6
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

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  2. Article ; Online: Pathogenic insights from genetic causes of autoinflammatory inflammasomopathies and interferonopathies.

    Lin, Bin / Goldbach-Mansky, Raphaela

    The Journal of allergy and clinical immunology

    2021  Volume 149, Issue 3, Page(s) 819–832

    Abstract: A number of systemic autoinflammatory diseases arise from gain-of-function mutations in genes encoding IL-1-activating inflammasomes or cytoplasmic nucleic acid sensors including the receptor and sensor STING and result in increased IL-1 and type I ... ...

    Abstract A number of systemic autoinflammatory diseases arise from gain-of-function mutations in genes encoding IL-1-activating inflammasomes or cytoplasmic nucleic acid sensors including the receptor and sensor STING and result in increased IL-1 and type I interferon production, respectively. Blocking these pathways in human diseases has provided proof-of-concept, confirming the prominent roles of these cytokines in disease pathogenesis. Recent insights into the multilayered regulation of these sensor pathways and insights into their role in amplifying the disease pathogenesis of monogenic and complex genetic diseases spurred new drug development targeting the sensors. This review provides insights into the pathogenesis and genetic causes of these "prototypic" diseases caused by gain-of function mutations in IL-1-activating inflammasomes (inflammasomopathies) and in interferon-activating pathways (interferonopathies) including STING-associated vasculopathy with onset in infancy, Aicardi-Goutieres syndrome, and proteasome-associated autoinflammatory syndromes that link activation of the viral sensors STING, "self" nucleic acid metabolism, and the ubiquitin-proteasome system to "type I interferon production" and human diseases. Clinical responses and biomarker changes to Janus kinase inhibitors confirm a role of interferons, and a growing number of diseases with "interferon signatures" unveil extensive cross-talk between major inflammatory pathways. Understanding these interactions promises new tools in tackling the significant clinical challenges in treating patients with these conditions.
    MeSH term(s) Humans ; Inflammasomes/genetics ; Interferon Type I/genetics ; Interleukin-1 ; Nucleic Acids ; Proteasome Endopeptidase Complex
    Chemical Substances Inflammasomes ; Interferon Type I ; Interleukin-1 ; Nucleic Acids ; Proteasome Endopeptidase Complex (EC 3.4.25.1)
    Language English
    Publishing date 2021-12-08
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Intramural ; Review
    ZDB-ID 121011-7
    ISSN 1097-6825 ; 1085-8725 ; 0091-6749
    ISSN (online) 1097-6825 ; 1085-8725
    ISSN 0091-6749
    DOI 10.1016/j.jaci.2021.10.027
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Book: Antizytokintherapie der rheumatoiden Arthritis

    Aringer, Martin / Goldbach-Mansky, Raphaela

    (UNI-MED Science)

    2009  

    Author's details [Martin Aringer. Unter Mitarb. von Raphaela Goldbach-Mansky ...]
    Series title UNI-MED Science
    Keywords Rheumatoide Arthritis ; Tumor-Nekrose-Faktor
    Subject Anti-TNF ; TNF ; Arthritis rheumatica ; Chronische Polyarthritis ; Chronisch-entzündlicher Gelenkrheumatismus ; Entzündlicher Gelenkrheumatismus ; PCP ; Polyarthritis rheumatica acuta ; Primär chronische Polyarthritis ; Progrediente chronische Polyarthritis ; Rheumatische Arthritis ; Rheumatische Gelenkerkrankung
    Language German
    Size 61 S. : Ill., graph. Darst.
    Edition 1. Aufl.
    Publisher UNI-MED Verl
    Publishing place Bremen u.a.
    Publishing country Germany
    Document type Book
    HBZ-ID HT016176633
    ISBN 978-3-8374-1151-5 ; 3-8374-1151-6
    Database Catalogue ZB MED Medicine, Health

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  4. Article ; Online: Human Autoinflammatory Diseases Mediated by NLRP3-, Pyrin-, NLRP1-, and NLRC4-Inflammasome Dysregulation Updates on Diagnosis, Treatment, and the Respective Roles of IL-1 and IL-18.

    Alehashemi, Sara / Goldbach-Mansky, Raphaela

    Frontiers in immunology

    2020  Volume 11, Page(s) 1840

    Abstract: Recent research has led to novel findings in inflammasome biology and genetics that altered the diagnosis and management of patients with autoinflammatory syndromes caused by NLRP3-, Pyrin-, NLRP1-, and NLRC4-inflammasomes and spurred the development of ... ...

    Abstract Recent research has led to novel findings in inflammasome biology and genetics that altered the diagnosis and management of patients with autoinflammatory syndromes caused by NLRP3-, Pyrin-, NLRP1-, and NLRC4-inflammasomes and spurred the development of novel treatments. The use of next-generation sequencing in clinical practice allows for rapid diagnosis and the detection of somatic mutations that cause autoinflammatory diseases. Clinical differences in patients with NLRP3, pyrin, and NLRP1 inflammasomopathies, and the constitutive elevation of unbound free serum IL-18 that predisposes to the development of macrophage activation syndrome (MAS) in patients with gain-of function mutations in
    MeSH term(s) Adaptor Proteins, Signal Transducing/immunology ; Apoptosis Regulatory Proteins/immunology ; CARD Signaling Adaptor Proteins/immunology ; Calcium-Binding Proteins/immunology ; Hereditary Autoinflammatory Diseases/immunology ; Humans ; Inflammasomes/immunology ; Inflammasomes/ultrastructure ; Interleukin-1/immunology ; Interleukin-18/immunology ; NLR Family, Pyrin Domain-Containing 3 Protein/immunology ; NLR Proteins ; Pyrin/immunology
    Chemical Substances Adaptor Proteins, Signal Transducing ; Apoptosis Regulatory Proteins ; CARD Signaling Adaptor Proteins ; Calcium-Binding Proteins ; IL18 protein, human ; Inflammasomes ; Interleukin-1 ; Interleukin-18 ; MEFV protein, human ; NLR Family, Pyrin Domain-Containing 3 Protein ; NLR Proteins ; NLRC4 protein, human ; NLRP1 protein, human ; NLRP3 protein, human ; Pyrin
    Language English
    Publishing date 2020-08-25
    Publishing country Switzerland
    Document type Journal Article ; Research Support, N.I.H., Intramural ; Review
    ZDB-ID 2606827-8
    ISSN 1664-3224 ; 1664-3224
    ISSN (online) 1664-3224
    ISSN 1664-3224
    DOI 10.3389/fimmu.2020.01840
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: JAK Inhibition in the Aicardi-Goutières Syndrome. Reply.

    Adang, Laura / Goldbach-Mansky, Raphaela / Vanderver, Adeline

    The New England journal of medicine

    2020  Volume 383, Issue 22, Page(s) 2191–2193

    MeSH term(s) Autoimmune Diseases of the Nervous System ; Humans ; Nervous System Malformations/genetics
    Language English
    Publishing date 2020-12-14
    Publishing country United States
    Document type Letter ; Comment
    ZDB-ID 207154-x
    ISSN 1533-4406 ; 0028-4793
    ISSN (online) 1533-4406
    ISSN 0028-4793
    DOI 10.1056/NEJMc2031081
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: Savvy About SAVI.

    Liptzin, Deborah R / Cole, Lyndsey / Schulte, Greg / Curtis, Donna / Curran, Megan L / Alehashemi, Sara / Goldbach-Mansky, Raphaela / Galambos, Csaba / Deutsch, Gail / Weinman, Jason P

    American journal of respiratory and critical care medicine

    2024  

    Language English
    Publishing date 2024-04-17
    Publishing country United States
    Document type Journal Article
    ZDB-ID 1180953-x
    ISSN 1535-4970 ; 0003-0805 ; 1073-449X
    ISSN (online) 1535-4970
    ISSN 0003-0805 ; 1073-449X
    DOI 10.1164/rccm.202309-1661IM
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article ; Online: Post-SARS-CoV-2 Vaccine Monitoring of Disease Flares in Autoinflammatory Diseases.

    Alehashemi, Sara / van Gelderen, Evelien / Rastegar, Andre / de Jesus, Adriana A / Goldbach-Mansky, Raphaela

    Journal of clinical immunology

    2022  Volume 42, Issue 4, Page(s) 732–735

    MeSH term(s) COVID-19/prevention & control ; COVID-19 Vaccines ; Hereditary Autoinflammatory Diseases/diagnosis ; Humans ; SARS-CoV-2 ; Symptom Flare Up ; Vaccination
    Chemical Substances COVID-19 Vaccines
    Language English
    Publishing date 2022-02-15
    Publishing country Netherlands
    Document type Letter ; Research Support, N.I.H., Intramural
    ZDB-ID 779361-3
    ISSN 1573-2592 ; 0271-9142
    ISSN (online) 1573-2592
    ISSN 0271-9142
    DOI 10.1007/s10875-022-01225-5
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article ; Online: Anakinra-associated renal amyloidosis.

    Nasr, Samih H / Alehashemi, Sara / Dasari, Surendra / Waldman, Meryl / Afzali, Behdad / Chiu, April / Bolanos, Jonathan / Goldbach-Mansky, Raphaela / McPhail, Ellen D

    Kidney international

    2024  Volume 105, Issue 2, Page(s) 395–396

    MeSH term(s) Humans ; Interleukin 1 Receptor Antagonist Protein/adverse effects ; Amyloidosis/chemically induced ; Amyloidosis/diagnosis ; Amyloidosis/drug therapy ; Treatment Outcome
    Chemical Substances Interleukin 1 Receptor Antagonist Protein
    Language English
    Publishing date 2024-01-19
    Publishing country United States
    Document type Case Reports
    ZDB-ID 120573-0
    ISSN 1523-1755 ; 0085-2538
    ISSN (online) 1523-1755
    ISSN 0085-2538
    DOI 10.1016/j.kint.2023.08.020
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  9. Article ; Online: Anakinra-Associated Amyloidosis.

    Alehashemi, Sara / Dasari, Surendra / de Jesus, Adriana A / Cowen, Edward W / Lee, Chia-Chia Richard / Goldbach-Mansky, Raphaela / McPhail, Ellen D

    JAMA dermatology

    2022  

    Language English
    Publishing date 2022-10-12
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2701761-8
    ISSN 2168-6084 ; 2168-6068
    ISSN (online) 2168-6084
    ISSN 2168-6068
    DOI 10.1001/jamadermatol.2022.2124
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Book ; Thesis: Eine co-stimulatiorische Rolle für CD28 in der Aktivierung von CD4 + T Lymphozyten durch Staphylokokken Enterotoxin B

    Goldbach-Mansky, Raphaela

    = A co-stimulatory role for CD28 in the activation of CD4 + T lymphocytes by staphylococcal enterotoxin B

    1992  

    Title variant A co-stimulatory role for CD28 in the activation of CD4 + T lymphocytes by staphylococcal enterotoxin B
    Author's details vorgelegt von: Raphaela Goldbach-Mansky
    Language English
    Size 63 S. : Ill., graph. Darst.
    Document type Book ; Thesis
    Thesis / German Habilitation thesis Witten/Herdecke, Univ., Diss., 1992
    Note Text engl.
    HBZ-ID HT006303187
    Database Catalogue ZB MED Medicine, Health

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