Results 51 to 60 of about 1,238 (171)
Single Cell Mechanics in Disease Progression
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
Emerin (EMD) and barrier to autointegration factor 1 (BANF1) each bind A-type lamins (LMNA) as fundamental components of nuclear lamina structure. Mutations in LMNA, EMD and BANF1 are genetically linked to many tissue-specific disorders including Emery ...
Tejas Dharmaraj +5 more
doaj +1 more source
Essential Role for Telomeric Repeat‐Binding Factor 2 in Cardiac Development and Function
This schematic illustrates a paradigm shift in telomere biology, demonstrating that Trf2 is a mandatory orchestrator of heart development and function through pathways distinct from its canonical telomere protective role. ABSTRACT Telomere repeat‐binding factor 2 (Trf2) is essential for protecting our telomeres.
Ali Hakim Shoushtari +11 more
wiley +1 more source
First Generation Proteolysis Targeting Chimeras (PROTACs) for the Treatment of Progeria
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
SUMO protease SENP6 protects the nucleus from hyperSUMOylation-induced laminopathy-like alterations
Summary: The small ubiquitin-like modifier (SUMO) protease SENP6 disassembles SUMO chains from cellular substrate proteins. We use a proteomic method to identify putative SENP6 substrates based on increased apparent molecular weight after SENP6 depletion.
Magda Liczmanska +6 more
doaj +1 more source
ABSTRACT Of the three types of cytoskeleton known in animals—actin, microtubules, and intermediate filaments—only actin and microtubules exist in plants. Both play important roles in cellular shaping, organelle movement, organization of the endomembrane system, and cell signaling.
Norman R. Groves +3 more
wiley +1 more source
Hutchinson-Gilford Progeria Syndrome—Current Status and Prospects for Gene Therapy Treatment
Hutchinson-Gilford progeria syndrome (HGPS) is one of the most severe disorders among laminopathies—a heterogeneous group of genetic diseases with a molecular background based on mutations in the LMNA gene and genes coding for interacting proteins.
Katarzyna Piekarowicz +3 more
doaj +1 more source
Lamin B1 safeguards the B cell genome and shapes lymphoma outcome
Abstract Lamin B1 is a structural component of the nuclear lamina that participates in genome organization and transcriptional control. During adaptive immune responses, B lymphocytes in germinal centers (GCs) undergo clonal expansion and programmed DNA damage at immunoglobulin loci, while simultaneously downregulating Lamin B1.
Filip Filipsky +12 more
wiley +1 more source
Despite matched clinical characteristics and LVEF, patients with Chagas cardiomyopathy exhibit a significantly higher arrhythmic burden compared to ischemic cardiomyopathy. This electro‐mechanical dissociation highlights the necessity of sudden death risk stratification strategies that extend beyond traditional ejection fraction thresholds.
Luis E. Echeverría +7 more
wiley +1 more source

