René Armand Dreifuss
In: Dados, Band 46, Heft 1, S. 195-197
ISSN: 0011-5258
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In: Dados, Band 46, Heft 1, S. 195-197
ISSN: 0011-5258
In: Schweizerische Ärztezeitung: SÄZ ; offizielles Organ der FMH und der FMH Services = Bulletin des médecins suisses : BMS = Bollettino dei medici svizzeri, Band 81, Heft 2, S. 71-71
ISSN: 1424-4004
In: Schweizerische Ärztezeitung: SÄZ ; offizielles Organ der FMH und der FMH Services = Bulletin des médecins suisses : BMS = Bollettino dei medici svizzeri, Band 83, Heft 19, S. 950-952
ISSN: 1424-4004
In: Neue Wege: der Geist des digitalen Kapitalismus ; Religion, Sozialismus, Kritik, Band 96, Heft 10, S. 302-304
In: Schweizerische Ärztezeitung: SÄZ ; offizielles Organ der FMH und der FMH Services = Bulletin des médecins suisses : BMS = Bollettino dei medici svizzeri, Band 83, Heft 28, S. 01500-01500
ISSN: 1424-4004
In: Neue Wege: der Geist des digitalen Kapitalismus ; Religion, Sozialismus, Kritik, Band 96, Heft 7-8, S. 198-206
In: Schweizerische Ärztezeitung: SÄZ ; offizielles Organ der FMH und der FMH Services = Bulletin des médecins suisses : BMS = Bollettino dei medici svizzeri, Band 83, Heft 19, S. 935-937
ISSN: 1424-4004
In: Allgemeine schweizerische Militärzeitschrift: ASMZ, Band 167, Heft 6, S. 5
ISSN: 0002-5925
In: Foro internacional: revista trimestral, Band 24, Heft 1, S. 109
ISSN: 0185-013X
In: Swiss Medical Forum ‒ Schweizerisches Medizin-Forum, Band 12, Heft 44
ISSN: 1424-4020
In: Schweizerische Ärztezeitung: SÄZ ; offizielles Organ der FMH und der FMH Services = Bulletin des médecins suisses : BMS = Bollettino dei medici svizzeri, Band 81, Heft 34, S. 1829-1837
ISSN: 1424-4004
In: European Journal of Sustainable Development: EJSD, Band 4, Heft 2
ISSN: 2239-6101
Mutations in the nuclear structural protein lamin A produce rare, tissue-specific diseases called laminopathies. The introduction of a human Emery-Dreifuss muscular dystrophy (EDMD)-inducing mutation into the C. elegans lamin (LMN-Y59C), recapitulates many muscular dystrophy phenotypes, and correlates with hyper-sequestration of a heterochromatic array at the nuclear periphery in muscle cells. Using muscle-specific emerin Dam-ID in worms, we monitored the effects of the mutation on endogenous chromatin. An increased contact with the nuclear periphery along chromosome arms, and an enhanced release of chromosomal centers, coincided with the disease phenotypes of reduced locomotion and compromised sarcomere integrity. The coupling of the LMN-Y59C mutation with the ablation of CEC-4, a chromodomain protein that anchors H3K9-methylated chromatin at the nuclear envelope (NE), suppressed the muscle-associated disease phenotypes. Deletion of cec-4 also rescued LMN-Y59C-linked alterations in chromatin organization and some changes in transcription. Sequences that changed position in the LMN-Y59C mutant, are enriched for E2F (EFL-2)-binding sites, consistent with previous studies suggesting that altered Rb-E2F interaction with lamin A may contribute to muscle dysfunction. In summary, we were able to counteract the dominant muscle-specific defects provoked by LMNA mutation by the ablation of a lamin-associated H3K9me anchor, suggesting a novel therapeutic pathway for EDMD. ; J.C.H. was supported by an FP7 Marie Curie Action Intra-European Fellowship and by a grant from the Foundation Suisse de Recherche sur les Maladies Musculaires. A.M. was funded by the same and by a Marie Heim-Vögtlin grant from the Swiss National Science Foundation. C.M.-J., R.R.-B., and P.A. thank the Spanish State Research Agency and the European Regional Development Fund (BFU2016-79313-P and MDM-2016- 0687) for support. This project received funding from the European Research Council under the European Union's Horizon 2020 Research and Innovation program (Epiherigans grant no. 743312 to S.M.G.). ; Peer reviewed
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