Institution
University of Kiel
Education•Kiel, Germany•
About: University of Kiel is a education organization based out in Kiel, Germany. It is known for research contribution in the topics: Population & Crystal structure. The organization has 27816 authors who have published 57114 publications receiving 2061802 citations. The organization is also known as: Christian Albrechts University & Christian-Albrechts-Universität zu Kiel.
Topics: Population, Crystal structure, Transplantation, Gene, Receptor
Papers published on a yearly basis
Papers
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TL;DR: Taking together, mutations in CTLA4 resulting inCTLA-4 haploinsufficiency or impaired ligand binding result in disrupted T and B cell homeostasis and a complex immune dysregulation syndrome.
Abstract: The protein cytotoxic T lymphocyte antigen-4 (CTLA-4) is an essential negative regulator of immune responses, and its loss causes fatal autoimmunity in mice. We studied a large family in which five individuals presented with a complex, autosomal dominant immune dysregulation syndrome characterized by hypogammaglobulinemia, recurrent infections and multiple autoimmune clinical features. We identified a heterozygous nonsense mutation in exon 1 of CTLA4. Screening of 71 unrelated patients with comparable clinical phenotypes identified five additional families (nine individuals) with previously undescribed splice site and missense mutations in CTLA4. Clinical penetrance was incomplete (eight adults of a total of 19 genetically proven CTLA4 mutation carriers were considered unaffected). However, CTLA-4 protein expression was decreased in regulatory T cells (Treg cells) in both patients and carriers with CTLA4 mutations. Whereas Treg cells were generally present at elevated numbers in these individuals, their suppressive function, CTLA-4 ligand binding and transendocytosis of CD80 were impaired. Mutations in CTLA4 were also associated with decreased circulating B cell numbers. Taken together, mutations in CTLA4 resulting in CTLA-4 haploinsufficiency or impaired ligand binding result in disrupted T and B cell homeostasis and a complex immune dysregulation syndrome.
660 citations
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University Hospital of Lausanne1, Harvard University2, Tel Aviv Sourasky Medical Center3, Tel Aviv University4, University of Illinois at Chicago5, Augsburg College6, University of Pittsburgh7, University of Virginia8, Northwestern University9, Claude Bernard University Lyon 110, Case Western Reserve University11, Medical College of Wisconsin12, University of Graz13, University of Kiel14, Cleveland Clinic15, Cornell University16, University of Hamburg17, Masaryk University18, Boston University19, University of Innsbruck20, University of Zurich21, Columbia University22, Tufts University23, NorthShore University HealthSystem24, Memorial Sloan Kettering Cancer Center25
TL;DR: No improvement in overall survival was demonstrated, however efficacy and activity with this chemotherapy-free treatment device appears comparable to chemotherapy regimens that are commonly used for recurrent glioblastoma.
659 citations
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TL;DR: In this article, an analysis of organic carbon, total nitrogen, and inorganically bound ammonium (exchangeable and fixed ammonium) in two oxic deep-sea sediment cores from the Central Pacific Ocean revealed insufficiently high inorganic ammonium contents of these sediments to explain the low C/N ratios.
658 citations
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TL;DR: The present paper gives an overview about the possibilities to use bacterial and fungal populations as an indicator for soil quality and the applicability of nematodes for the determination of soil health.
658 citations
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TL;DR: Impaired autophagic degradation contributes to the pathogenesis of several human diseases including lysosomal storage disorders and muscle diseases, and has been shown to possess important housekeeping and quality control functions that contribute to health and longevity.
657 citations
Authors
Showing all 28103 results
Name | H-index | Papers | Citations |
---|---|---|---|
Stefan Schreiber | 178 | 1233 | 138528 |
Jun Wang | 166 | 1093 | 141621 |
William J. Sandborn | 162 | 1317 | 108564 |
Jens Nielsen | 149 | 1752 | 104005 |
Tak W. Mak | 148 | 807 | 94871 |
Annette Peters | 138 | 1114 | 101640 |
Severine Vermeire | 134 | 1086 | 76352 |
Peter M. Rothwell | 134 | 779 | 67382 |
Dusan Bruncko | 132 | 1042 | 84709 |
Gideon Bella | 129 | 1301 | 87905 |
Dirk Schadendorf | 127 | 1017 | 105777 |
Neal L. Benowitz | 126 | 792 | 60658 |
Thomas Schwarz | 123 | 701 | 54560 |
Meletios A. Dimopoulos | 122 | 1371 | 71871 |
Christian Weber | 122 | 776 | 53842 |