Results 71 to 80 of about 1,703 (200)

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, Jongmin Lee, Kyungtae Lim, Dong‐Hwee Kim   +3 more
wiley   +1 more source

Dateset related to article "Cytokines as potential biomarkers of disease clinical course in muscular dystrophies "

, 2021
This database includes a comprehensive profile of circulating pro- and anti-inflammatory molecules (cytokines, chemokines and growth factors) obtained from a group of 53 patients with muscular laminopathy, 10 with non-muscular laminopathy, 22 with other ...
Lattanzi, Giovanna, Cappelletti, Cristina, Maggi, Lorenzo   +2 more
core   +1 more source

Essential Role for Telomeric Repeat‐Binding Factor 2 in Cardiac Development and Function

FASEB BioAdvances, Volume 8, Issue 7, July 2026.
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, Aysenure Danis, Michael Wayne Stoner, Jeffrey Baust, Andrea Sebastiani, Paramesha Bugga, Janet R. Manning, Bellina A. S. Mushala, Nisha Bhattarai, Imad Al Ghouleh, Iain Scott, Maryam Sharifi‐Sanjani   +11 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, Marcelino Telechea, Daniel Fernández, Alba García‐Martín, Ángeles Canales, Silvia Ortega‐Gutiérrez   +5 more
wiley   +1 more source

Hutchinson-Gilford Progeria Syndrome—Current Status and Prospects for Gene Therapy Treatment

Cells, 2019
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, Magdalena Machowska, Volha Dzianisava, Ryszard Rzepecki   +3 more
doaj   +1 more source

Heterozygous lamin B1 and lamin B2 variants cause primary microcephaly and define a novel laminopathy [PDF]

, 2020
PURPOSE: Lamins are the major component of nuclear lamina, maintaining structural integrity of the nucleus. Lamin A/C variants are well established to cause a spectrum of disorders ranging from myopathies to progeria, termed laminopathies.
Martin, CA, Smith, KR, Stevens, HE, Lopez, FJ, Patch, C, Boustred, CR, Zarowiecki, M, Smith, SC, Elgar, G, Odhams, CA, Tregidgo, C, Tucci, A, Greville-Heygate, S, Jackson, AP, Need, AC, Sosinsky, A, Boissiere, JM, Riesgo-Ferreiro, P, Stuckey, A, Sultana, R, Daugherty, LC, Wood, SM, Suri, M, Holman, JE, Marsh, JA, Foulger, RE, Sawant, K, McDonagh, EM, Blyth, M, Jackson, R, Brittain, H, Craig, CEH, Bleda, M, Donnelly, D, Rogers, T, Caulfield, MJ, Scott, RH, Henderson, S, Murugaesu, N, Boardman-Pretty, F, Siddiq, A, Lachlan, K, Leong, IUS, Perez-Gil, D, Pereira, MB, Witkowska, K, Williams, E, Hackett, JM, Rea, G, Ambrose, JC, Jones, LJ, Greene, P, Furió-Tarí, P, Watters, SA, de Burca, A, Hubbard, TJP, Kayikci, M, Maleady-Crowe, F, Mueller, M, Rahim, T, Welland, MJ, Devereau, A, Sieghart, A, Lawson, K, Leigh, SEA, Fowler, T, Orioli, A, Lahnstein, L, Mason, J, Kousathanas, A, Cox, H, Savage, K, Smedley, D, Rendon, A, Walsh, E, Thompson, SR, Moutsianas, L, Polychronopoulos, D, Hamblin, A, Quigley, AJ, Kasperaviciute, D, Baple, EL, Chan, GC, Harrison, V, Ibáñez, K, Arumugam, P, Ryten, M, Spooner, W, Greenhalgh, L, Giess, A, Tanguy, M, McKenna, C, Halai, D, Parry, DA, Robertson, L, Thomas, ERA, Pullinger, J   +96 more
core   +1 more source

Nuclear Entanglement: New Insights Into the Role of Cytoskeleton and Nucleoskeleton in Plant Nuclear Function

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, Katelyn Amstutz, Lily A. Schumacher, Iris Meier   +3 more
wiley   +1 more source

Differences in cardiac phenotype and natural history of laminopathies with and without neuromuscular onset

Orphanet Journal of Rare Diseases, 2019
Objective To investigate differences in cardiac manifestations of patients affected by laminopathy, according to the presence or absence of neuromuscular involvement at presentation.
Raffaello Ditaranto, Giuseppe Boriani, Mauro Biffi, Massimiliano Lorenzini, Maddalena Graziosi, Matteo Ziacchi, Ferdinando Pasquale, Giovanni Vitale, Alessandra Berardini, Rita Rinaldi, Giovanna Lattanzi, Luciano Potena, Sofia Martin Suarez, Maria Letizia Bacchi Reggiani, Claudio Rapezzi, Elena Biagini   +15 more
doaj   +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, Katarina B. Chapman, Johannes Bloehdorn, Oscar Maiques, Abigail Lee, Andrew Clear, Jun Wang, John Gribben, Michael Hausmann, Christoph Cremer, Andrejs Braun, Marta C. Sallan, Tanya Klymenko   +12 more
wiley   +1 more source

Sterile inflammation in laminopathies

European Journal of Cell Biology
Sterile inflammation, an immune response triggered in the absence of pathogens, plays a key role in various chronic diseases, including aging-related disorders, cancer, and autoimmune conditions. This process is driven by damage-associated molecular patterns, such as self-DNA in the cytosol, which activate innate immune pathways and contribute to ...
Rafael Cancado de Faria, Susana Gonzalo
openaire   +2 more sources