Results 51 to 60 of about 3,734 (156)
Nuclear dysfunction in aging and neurodegeneration
Alzheimer's &Dementia, Volume 22, Issue 7, July 2026.Abstract Neurodegenerative diseases are characterized by a loss of neuronal function and structure, often in a region‐specific manner. Multiple factors contribute to neuronal dysfunction and death, including pathogenic protein buildup, protein mislocalization, and inflammation. Despite extensive research, the common mechanisms driving neurodegeneration
Abbigael Aday +7 more
wiley +1 more source
Single Cell Mechanics in Disease Progression
Small Science, Volume 6, Issue 7, July 2026.Cells transmit distinct mechanical forces through ECM adhesion and cell–cell junctions and actomyosin‐generated traction forces are transmitted to the substrate through integrin‐based focal adhesions. Mechanical signals are further transmitted to the nucleus via the LINC complex, connecting the cytoskeleton to the nuclear lamina. These forces integrate
Sabin Kim +3 more
wiley +1 more source
Induced Pluripotent Stem Cells to Study Mechanisms of Laminopathies: Focus on Epigenetics
Frontiers in Cell and Developmental Biology, 2018 Laminopathies are a group of rare degenerative disorders that manifest with a wide spectrum of clinical phenotypes, including both systemic multi-organ disorders, such as the Hutchinson-Gilford Progeria Syndrome (HGPS), and tissue-restricted diseases ...
Silvia Crasto +3 more
doaj +1 more source
, 2012 The laminopathies are a group of rare diseases characterized by a vast range of phenotypic alterations, due to mutations in lamin A and C or other nuclear envelope proteins.
Nadir M Maraldi, Giovanna Lattanzi
core +1 more source
Frontiers in Cardiovascular Medicine, 2022 Pathogenic variants in the LMNA gene are known to cause laminopathies, a broad range of disorders with different clinical phenotypes. LMNA genetic variants lead to tissue-specific pathologies affecting various tissues and organs. Common manifestations of
Kseniya Perepelina +10 more
doaj +1 more source
First Generation Proteolysis Targeting Chimeras (PROTACs) for the Treatment of Progeria
Advanced Science, Volume 13, Issue 32, 9 June 2026.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
Genotype-phenotype correlations in laminopathies: how does fate translate?
, 2010 A-type laminopathies are a group of diseases resulting from mutations in the intermediate filament proteins lamin A and C (both encoded by the LMNA gene), but for which the pathogenic mechanisms are little understood.
Scharner, Juergen +3 more
core +1 more source
Laminopathies: Too Much SUN Is a Bad Thing [PDF]
, 2012 SummarySUN proteins accelerate the pathological progression of laminopathies. Although the mechanisms remain to be elucidated, an intriguing possibility is that high levels of SUN proteins lead to a hyperactive DNA damage ...
Starr, Daniel A.
core +1 more source
The responsive nucleus: morphological signatures of cellular state
NucleusNuclear morphology is increasingly recognized as an integrative indicator of cellular state across diverse physiological and pathological conditions.
Alejandro Pérez-Venteo +3 more
doaj +1 more source
Separation of Coiled-Coil Structures in Lamin A/C Is Required for the Elongation of the Filament
Cells, 2020 Intermediate filaments (IFs) commonly have structural elements of a central α-helical coiled-coil domain consisting of coil 1a, coil 1b, coil 2, and their flanking linkers.
Jinsook Ahn +5 more
doaj +1 more source