Results 51 to 60 of about 3,036 (198)

miR‐140‐5p Overexpression Contributes to Oxidative Stress and Mitochondrial Dysfunction in Hutchinson‐Gilford Progeria Syndrome Fibroblasts Through NRF2 Pathway

Aging Cell, Volume 24, Issue 12, December 2025.
This study identifies a novel molecular mechanism involving miR‐140‐5p that contributes to the pathogenesis of HGPS. By decreasing NRF2 expression, miR‐140‐5p overexpression results in downregulation of the NRF2/KEAP1/HO‐1 antioxidant pathway in HGPS fibroblasts, leading to increased oxidative stress and mitochondrial dysfunction, two hallmarks of ...
Léa Toury, Diane Frankel, Sara Nael, Mario Abaji, Léa Le Goff, Marie Basset, Coraline Airault, Bertrand Vernay, Elva María Novoa‐del‐Toro, Catherine Bartoli, Anaïs Baudot, Frederique Magdinier, Elise Kaspi, Patrice Roll   +13 more
wiley   +1 more source

LMNA mutation leads to cardiac sodium channel dysfunction in the Emery-Dreifuss muscular dystrophy patient

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, Kseniya Perepelina, Anastasia Zaytseva, Anastasia Zaytseva, Aleksandr Khudiakov, Irina Neganova, Elena Vasichkina, Anna Malashicheva, Anna Malashicheva, Anna Kostareva, Anna Kostareva   +10 more
doaj   +1 more source

SENP6 Maintains Mitochondrial Homeostasis by Regulating Mitochondrial Protein Import Through deSUMOylation of TOM40

Advanced Science, Volume 12, Issue 40, October 27, 2025.
SUMOylation regulates mitochondrial processes, but its impact on protein import remains unclear. TOM40 is identified, a mitochondrial outer membrane channel protein, as a substrate of deSUMOylase SENP6. TOM40 SUMOylation disrupts outer membrane complex assembly, inhibits protein import, and compromises mitochondrial homeostasis.
Liubing Hu, Jianshuang Li, Haolin Guo, Lei Su, Peina Dong, Juan Huang, Yanyan Liu, Xinjie Liu, Zhenhuan Luo, Wei Xiong, Zhenyu Ju, Qinghua Zhou, Hao Wang, Wenjun Wang   +13 more
wiley   +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, Soyeon Jeong, So-Mi Kang, Inseong Jo, Bum-Joon Park, Nam-Chul Ha   +5 more
doaj   +1 more source

Nuclear Lamins: Key Proteins for Embryonic Development

Biology, 2022
Lamins are essential components of the nuclear envelope and have been studied for decades due to their involvement in several devastating human diseases, the laminopathies.
Jasper Chrysolite Paul, Helena Fulka
doaj   +1 more source

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

Laminopathies: what can humans learn from fruit flies

Cellular & Molecular Biology Letters, 2018
Lamin proteins are type V intermediate filament proteins (IFs) located inside the cell nucleus. They are evolutionarily conserved and have similar domain organization and properties to cytoplasmic IFs. Lamins provide a skeletal network for chromatin, the
Marta Pałka, Aleksandra Tomczak, Katarzyna Grabowska, Magdalena Machowska, Katarzyna Piekarowicz, Dorota Rzepecka, Ryszard Rzepecki   +6 more
doaj   +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

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

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