Myopathic lamin mutations impair nuclear stability in cells and tissue and disrupt nucleo-cytoskeletal coupling [PDF]
, 2017 Lamins are intermediate filament proteins that assemble into a meshwork underneath the inner nuclear membrane, the nuclear lamina. Mutations in the LMNA gene, encoding lamins A and C, cause a variety of diseases collectively called laminopathies.
Dialynas, George +8 more
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Nuclear Lamins in Cell Regulation and Disease [PDF]
Cold Spring Harbor Symposia on Quantitative Biology, 2010 The nuclear lamins are type V intermediate filament proteins that form meshworks at the inner aspect of the nuclear envelope and are also present throughout the nuclear interior. Through these meshwork structures, lamins regulate the shape, size, and mechanical properties of the nucleus.
T, Shimi +3 more
openaire +2 more sources
Advanced Science, EarlyView.Grancalcin (GCA), a myeloid‐derived protein, is enriched in gingival tissues of periodontitis patients and mouse models. Through interactions with CD44 and activation of MYH9, GCA promotes NF‐κB signaling and exacerbates periodontal inflammation and bone loss.
Min Zhou +6 more
wiley +1 more source
Expression of proliferation-dependent antigens during cellular ageing of normal and progeroid human fibroblasts [PDF]
, 1994 Normal human fibroblasts display a limited lifespan in culture, which is due to a steadily decreasing fraction of cells that are able to proliferate.
Faragher, RA +3 more
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Roles of Cross-Membrane Transport and Signaling in the Maintenance of Cellular Homeostasis [PDF]
, 2016 Organelles allow specialized functions within cells to be localized, contained and independently regulated. This separation is oftentimes achieved by selectively permeable membranes, which enable control of molecular transport, signaling between ...
Inchul Cho, Joe Swift, Mark R. Jackson
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PSMA8‐Containing 20S Proteasome Regulates Spermiogenesis and Male Fertility
Advanced Science, EarlyView.PSMA8 assembles s20S proteasome that degrades specific substrates in elongating spermatids. Degradation of s20S‐substrates activates translation of FXR1‐target mRNAs, which are essential for mitochondrial sheath formation and sperm morphogenesis.
Huiwen Cao +7 more
wiley +1 more source
The Nuclear Envelope Lamina Network Has Elasticity and a Compressibility Limit Suggestive of a Molecular Shock Absorber [PDF]
, 2004 Author Posting © Journal of Cell Science, 2004. This article is posted here by permission of Journal of Cell Science for personal use, not for redistribution.
Kahn, Samuel
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Nucleus, 2013 Anomalies in the three-dimensional shape of the nucleus are associated with a number of genetic diseases. These shape distortions include lobulated structures, with localized bulges referred to as nuclear blebs. Blebbing can result from mutations in genes encoding lamin intermediate filaments that form the lamin cortex, a thin meshwork lining the ...
Chase P, Broedersz +1 more
openaire +2 more sources
First Generation Proteolysis Targeting Chimeras (PROTACs) for the Treatment of Progeria
Advanced Science, EarlyView.We report the first PROTACs designed to degrade progerin, introducing a novel therapeutic approach for progeria. The best compound, UCM‐18142, significantly reduces progerin levels and improves key disease phenotypes in patient‐derived cells and in the LmnaG609G/G609G mouse model, paving the way for new treatment strategies targeting the root cause of ...
Jon Macicior‐Michelena +5 more
wiley +1 more source
The truncated prelamin A in Hutchinson-Gilford progeria syndrome alters segregation of A-type and B-type lamin homopolymers. [PDF]
, 2006 Hutchinson-Gilford progeria syndrome (HGPS) is a dominant autosomal premature aging syndrome caused by the expression of a truncated prelamin A designated progerin.
Buendia, Brigitte +5 more
core +1 more source