Results 1 to 10 of about 2,404 (88)
Genetic and Pathophysiological Basis of Cardiac and Skeletal Muscle Laminopathies [PDF]
GenesGirish C Melkani +2 more
exaly +2 more sources
Advanced Intelligent Systems, EarlyView.HTFC gets 3D refractive index tomograms of flowing cells. Label‐free monocytes are engineered to express patterns of cytoplasmic vacuoles. From the tomogram, an efficient dimensionality reduction is operated. Interpretable features are extracted to classify the expression severity of phenotypes coexisting in each cell, visually represented by a seven ...
Marika Valentino +9 more
wiley +1 more source
The FEBS Journal, EarlyView.Chd4/NuRD and ThPOK cooperate to maintain transcriptional repression and nuclear organization in adult cardiomyocytes. Chd4 loss reduces miR‐150‐5p, relieving repression of Sprr1a, while ThPOK loss further enhances Sprr1a activation, possibly through altered chromatin–lamina interactions.
Fadoua El Abdellaoui‐Soussi +12 more
wiley +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
Cytoskeleton, Volume 83, Issue 6, Page 354-375, June 2026.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
Lamin B1 safeguards the B cell genome and shapes lymphoma outcome
HemaSphere, Volume 10, Issue 6, June 2026.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
Journal of Arrhythmia, Volume 42, Issue 3, June 2026.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
Dynamic expression of lamin B1 during adult neurogenesis in the vertebrate brain
Developmental Dynamics, Volume 255, Issue 2, Page 187-208, February 2026.Abstract Background In mammals, specific brain regions such as the dentate gyrus (DG) of the hippocampus and the subventricular zone (SVZ) of the lateral ventricles harbor adult neural stem/progenitor cells (ANSPCs) that give rise to new neurons and contribute to structural and functional brain plasticity.
Diana Zhilina +12 more
wiley +1 more source
Journal of Cachexia, Sarcopenia and Muscle, Volume 17, Issue 1, February 2026.ABSTRACT Background Emery–Dreifuss muscular dystrophy (EDMD) is a progressive genetic myopathy that mainly affects the muscles used for movement (skeletal muscles) and the heart (cardiac muscles). The disease is frequently associated with mutations in genes encoding nuclear envelope proteins, most notably LMNA, which encodes lamin A—a critical ...
So‐mi Kang +11 more
wiley +1 more source
Informing Dose for Pediatric Rare Diseases—A Survey of Recent Orphan Drugs Approvals
Clinical and Translational Science, Volume 19, Issue 2, February 2026.ABSTRACT Collectively, pediatric rare diseases affect millions of children worldwide. Yet, treatment options are limited. Dose selection presents unique challenges in pediatric rare disease drug development. Traditional dose‐finding approaches are impractical for these populations, and conventional pediatric dosing methods like exposure matching face ...
Elimika Pfuma Fletcher +6 more
wiley +1 more source