Results 91 to 100 of about 2,917 (255)

The Inner Nuclear Membrane Has a Unique Lipid Signature

BioEssays, Volume 47, Issue 10, October 2025.
The inner nuclear membrane (INM) has a distinct lipid profile, most notably characterized by high enrichment of phosphatidylserine (PS). In this review, Yang Niu and Tamas Balla summarize recent advances in elucidating the INM's lipid composition and related metabolic pathways, while elaborating further on their potential functional roles.
Yang Niu, Tamas Balla
wiley   +1 more source

Emerging perspectives on laminopathies

Cell Health and Cytoskeleton, 2016
Laminopathies are a group of inherited disorders caused by mutations in the lamin A/C gene, and can affect diverse organs or tissues, or can be systemic, causing premature aging. In the present review, we report on the composition and structure of the nuclear lamina and the role of lamins in nuclear mechanics and their involvement in human diseases ...
Lattanzi, Giovanna, Benedetti, Sara, D’Apice, Maria Rosaria, Maggi, Lorenzo, Carboni, Nicola, Scarano, Emanuela, POLITANO, Luisa   +6 more
openaire   +5 more sources

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

Concise Review: Plasma and Nuclear Membranes Convey Mechanical Information to Regulate Mesenchymal Stem Cell Lineage [PDF]

, 2016
Numerous factors including chemical, hormonal, spatial, and physical cues determine stem cell fate. While the regulation of stem cell differentiation by soluble factors is well-characterized, the role of mechanical force in the determination of lineage ...
Fuchs, Robyn K., Rubin, Janet, Thompson, William R., Uzer, Gunes   +3 more
core   +1 more source

Atrial‐secondary tricuspid regurgitation: a better prognosis in early stage heart failure, but not in late stage

ESC Heart Failure, Volume 12, Issue 5, Page 3435-3443, October 2025.
Atrial TR as more favourable prognosis except in advanced patients. Abstract Background and Aims Although the classification of secondary tricuspid regurgitation (STR) by atrial or ventricular aetiology (A‐STR or V‐STR) carries prognostic importance, the confounding effects of New York Heart Association (NYHA) class have not yet been elucidated.
Corentin Bourg, K. Charlotte Lee Frost, Augustin Coisne, Elizabeth Curtis, Guillaume L'official, Yoan Lavie‐Badie, Léo Lemarchand, Julien Dreyfus, Emmanuel Oger, Erwan Donal   +9 more
wiley   +1 more source

Cardiac involvement in laminopathies [PDF]

Orphanet Journal of Rare Diseases, 2015
Lamin A/C gene mutations can be associated with myocardial diseases, usually characterized by dilated cardiomyopathy and/or arrhythmic disorders. Phenotypic penetrance is age-related but expression is extremely heterogeneous, so that muscular and arrhythmic disease can be present in combination in the same patient, or one phenotypic manifestation can ...
Boriani, Giuseppe, Biagini, Elena, Wahbi, Karim, Duboc, Denis   +3 more
openaire   +1 more source

Vascular disease modeling using induced pluripotent stem cells: Focus in Hutchinson-Gilford Progeria Syndrome [PDF]

, 2016
Transparency document related to this article can be found online at http://dx.doi.org/10.1016/j.bbrc.2015.10.014Induced pluripotent stem cells (iPSCs) represent today an invaluable tool to create disease cell models for modeling and drug screening ...
Ferreira, L., Pereira, P., Praça, C., Rosa, S. C.   +3 more
core   +1 more source

cTAGE5/MEA6 Regulates LBR Localization to Maintain Nuclear Envelope Integrity and Safeguard Against Aging

Aging Cell, Volume 24, Issue 10, October 2025.
cTAGE5 regulates LBR trafficking between the ER and the nucleus membrane to maintain the integrity of both the ER and the nuclear envelope. cTAGE5 KO results in LBR retention in the ER, reduced stability, and subsequent disruption of nuclear envelope integrity. ABSTRACT cTAGE5/MEA6 plays a pivotal role in COPII complex assembly, ER‐to‐Golgi trafficking,
Yaqing Wang, Pengyu Sun, Fuqiang Yang, Tiantian Ma, Junwan Fan, Lei Shi, Nan Li, Fei Gao, Kangmin He, Zhiheng Xu   +9 more
wiley   +1 more source

LMNA-Cardiomyopathy in Emery-Dreifuss Muscular Dystrophy

Архивъ внутренней медицины
Emery-Dreifuss muscular dystrophy is a rare disease resulting from a genetic defect in nuclear envelope proteins, most commonly in emerin and lamin A/C.
E. V. Resnik, A. A. Kovaleva, M. Kh. Shurdumova, D. E. Emelyanovich, A. P. Smirnov, V. Y. Voinova   +5 more
doaj   +1 more source

Emery-Dreifuss muscular dystrophy: the most recognizable laminopathy

Folia Neuropathologica, 2016
Emery-Dreifuss muscular dystrophy (EDMD), a rare inherited disease, is characterized clinically by humero-peroneal muscle atrophy and weakness, multijoint contractures, spine rigidity and cardiac insufficiency with conduction defects.
Agnieszka Madej-Pilarczyk, Andrzej Kochański   +1 more
doaj   +1 more source