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Emery–Dreifuss muscular dystrophy (EDMD), caused by mutations in genes encoding nuclear envelope proteins, is clinically characterized by muscular dystrophy, early joint contracture, and life-threatening cardiac abnormalities.
Eiji Wada +4 more
doaj +1 more source
Ebola virus (EBOV) VP24 protein is a nucleocapsid-associated protein that inhibits interferon (IFN) gene expression and counteracts the IFN-mediated antiviral response, preventing nuclear import of signal transducer and activator of transcription 1 ...
Santiago Vidal +13 more
doaj +1 more source
Centrosome maturation requires YB-1 to regulate dynamic instability of microtubules for nucleus reassembly [PDF]
Microtubule formation from the centrosome increases dramatically at the onset of mitosis. This process is termed centrosome maturation. However, regulatory mechanisms of microtubule assembly from the centrosome in response to the centrosome maturation ...
Asaka Masamitsu N. +5 more
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Emerin hooks centrosome to nucleus [PDF]
Cells lacking emerin, one of the proteins whose loss causes a form of muscular dystrophy, cannot hook their centrosome to the nucleus, show Salpingidou et al. The lost linkage might weaken contractile cells, including those diseased muscles. Figure 1 Centrosomes (arrowheads) stray from the nucleus if emerin is missing (bottom). Besides
openaire +1 more source
Nuclear envelope laminopathies: evidence for developmentally inappropriate chromatin-nuclear envelope interactions [PDF]
During terminal differentiation of cells, there is typically a transition of the nuclear envelope from the Lamin B protein to Lamin A/C proteins.
Eric Hoffman +3 more
core +2 more sources
Emerin self‐assembly mechanism: role of the LEM domain [PDF]
At the nuclear envelope, the inner nuclear membrane protein emerin contributes to the interface between the nucleoskeleton and the chromatin. Emerin is an essential actor of the nuclear response to a mechanical signal. Genetic defects in emerin cause Emery–Dreifuss muscular dystrophy. It was proposed that emerin oligomerization regulates nucleoskeleton
Samson, Camille +12 more
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Lamin A/C and Emerin depletion impacts chromatin organization and dynamics in the interphase nucleus
Background Nuclear lamins are type V intermediate filament proteins that maintain nuclear structure and function. Furthermore, Emerin - an interactor of Lamin A/C, facilitates crosstalk between the cytoskeleton and the nucleus as it also interacts with ...
Devika Ranade +4 more
doaj +1 more source
Mouse models of nesprin-related diseases [PDF]
Nesprins (nuclear envelope spectrin repeat proteins) are a family of multi-isomeric scaffolding proteins. Nesprins form the LInker of Nucleoskeleton-and-Cytoskeleton (LINC) complex with SUN (Sad1p/UNC84) domain-containing proteins at the nuclear envelope,
Akinrinade +93 more
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Distribution of emerin during the cell cycle
Human emerin is a nuclear membrane protein that is lost or altered in patients with Emery-Dreifuss muscular dystrophy (EMD). While the protein is expressed in the majority of human tissues analyzed, the pathology predominates in cardiac and skeletal muscles of patients with EMD.
M C, Dabauvalle +5 more
openaire +2 more sources
Nuclear Envelope, Nuclear Lamina, and Inherited Disease [PDF]
The nuclear envelope is composed of the nuclear membranes, nuclear lamina, and nuclear pore complexes. In recent years, mutations in nuclear-envelope proteins have been shown to cause a surprisingly wide array of inherited diseases.
Courvalin, Jean-Claude, Worman, Howard,
core +4 more sources

