Results 51 to 60 of about 24,019 (217)

Recent Advances in Animal and Human Pluripotent Stem Cell Modeling of Cardiac Laminopathy [PDF]

, 2016
published_or_final_versio
Jiang, Y, Lai, KWH, Lau, VYM, Lee, YK, Ng, KM, RAN, X, Siu, DCW, Tse, HF   +7 more
core   +1 more source

Lamin A Δexon9 mutation leads to telomere and chromatin defects but not genomic instability [PDF]

, 2013
Over 300 mutations in the LMNA gene, encoding A-type lamins, are associated with 15 human degenerative disorders and premature aging syndromes. Although genomic instability seems to contribute to the pathophysiology of some laminopathies, there is ...
Das, Arindam, Gonzalez-Suarez, Ignacio, Gonzalo, Susana, Grotsky, David A, Kennedy, Brian K, Kreienkamp, Ray, Neumann, Martin A, Redwood, Abena B, Stewart, Colin L   +8 more
core   +2 more sources

Human Atlas of Tooth Decay Progression: Identification of Cellular Mechanisms Driving the Switch from Dental Pulp Repair Toward Irreversible Pulpitis

Advanced Science, EarlyView.
Tooth decay progression transforms the dental pulp response from repair to fibrosis. At early stages, stromal cells reprogram to repair the extra cellular matrix (ECM), blood vessels, and nerves, remodel and grow, keeping repair possible. In advanced decay, hypoxia, and vessel regression, in complement with an immune switch, fuel nerve degeneration and
Hoang Thai Ha, Sofya Kosmynina, Amandine Verocq, Keremsah Ozen, Ines Tekia, Hugo Bussy, Marie Ramirez, Dima Sabbah, Chloe Goemans, Valerie Vandenbempt, Esteban N. Gurzov, Sumeet Pal Singh, Nicolas Baeyens   +12 more
wiley   +1 more source

Generation of a human induced pluripotent stem cell line derived from a patient with dilated cardiomyopathy carrying LMNA nonsense mutation

Stem Cell Research, 2022
Dilated cardiomyopathy (DCM) is a refractory heart disease characterized by dilation of the left ventricle and systolic dysfunction. LMNA, the gene encoding lamin A/C (a nuclear envelope protein), is the second leading causative gene associated with ...
Yuzuno Shimoda, Nobuyuki Murakoshi, Haruka Mori, DongZhu Xu, Kazuko Tajiri, Yasuko Hemmi, Iori Sato, Michiya Noguchi, Yukio Nakamura, Yohei Hayashi, Masaki Ieda   +10 more
doaj   +1 more source

Nuclear rupture at sites of high curvature compromises retention of DNA repair factors. [PDF]

, 2018
The nucleus is physically linked to the cytoskeleton, adhesions, and extracellular matrix-all of which sustain forces, but their relationships to DNA damage are obscure. We show that nuclear rupture with cytoplasmic mislocalization of multiple DNA repair
Alshareeda, Alvey, Andrea J. Liu, Broers, Burma, Buxboim, Buxboim, Capo-chichi, Chang, Charlotte R. Pfeifer, Coffinier, Cory M. Alvey, Cumming, Darzynkiewicz, Das, Dazhen Liu, de la Rosa, De Vos, Denais, Dennis E. Discher, Dingal, Docheva, Elosegui-Artola, Fong, Frigault, Grundy, Gudmundsdottir, Harada, Harding, Hatch, Hategan, Hernandez, Irena L. Ivanovska, Irianto, Ivanovska, Jerome Irianto, Jiazheng Ji, Kaspi, Kuangzheng Zhu, Lammerding, Larrieu, Levental, Lucas Smith, Nuciforo, Odde, Oh, Raab, Rachel R. Bennett, Roger A. Greenberg, Saez, Samudram, Sangkyun Cho, Schneider, Seluanov, Shin, Soubeyrand, Staszewski, Swift, Takaki, Tamiello, Tang, Turgay, Wang, Yuntao Xia, Álvarez-Quilón   +64 more
core   +2 more sources

The Mitochondrial Guardian α‐Amyrin Mitigates Alzheimer's Disease Pathology via Modulation of the DLK‐SARM1‐ULK1 Axis

Advanced Science, EarlyView.
Dietary habits play a key role in chronic diseases, and higher annual consumption of fruit and vegetable may lower risk of dementia. Artificial intelligence predicts the lipid‐like compound α‐Amyrin (αA) from plants with edible peels as a drug candidate against Alzheimer's disease.
Shu‐Qin Cao, Juan Ignacio Jiménez‐Loygorri, Yunguang Qiu, You Jung Kang, Khanh Van Do, Annabel E. Smith, Junjie Huang, Jun‐ping Pan, Lipeng Mao, Ang Li, Hangge Yang, Yahyah Aman, Maria Jose Donate Lagartos, Sofie Hindkjær Lautrup, Anbin Chen, Kristina Xiao Liang, Hailong Zhang, Juan Yi, Xurui Jin, Tin Cho Cheung, Olympia Apokotou, Florentia Papastefanaki, Rebecca Matsas, William A. McEwan, Feixiong Cheng, Hansang Cho, Guobing Chen, Huanxing Su, Konstantinos Palikaras, Oscar Junhong Luo, Qian Hua Zhao, Ding Ding, Cornelia van Duijn, Nektarios Tavernarakis, Patricia Boya, Tewin Tencomnao, Evandro F. Fang   +36 more
wiley   +1 more source

Creatine and l-carnitine attenuate muscular laminopathy in the LMNA mutation transgenic zebrafish

Scientific Reports
Lamin A/C gene (LMNA) mutations contribute to severe striated muscle laminopathies, affecting cardiac and skeletal muscles, with limited treatment options.
Shao-Wei Pan, Horng-Dar Wang, He-Yun Hsiao, Po-Jui Hsu, Yung-Che Tseng, Wen-Chen Liang, Yuh-Jyh Jong, Chiou-Hwa Yuh   +7 more
doaj   +1 more source

Phenotype-Genotype Analysis of Chinese Patients with Early-Onset LMNA-Related Muscular Dystrophy. [PDF]

PLoS ONE, 2015
This study aimed to analyze the correlation between the phenotype and genotype of Chinese patients with early-onset lamin A (LMNA)-related muscular dystrophy (MD).
Dandan Tan, Haipo Yang, Yun Yuan, Carsten Bonnemann, Xingzhi Chang, Shuang Wang, Yuchen Wu, Xiru Wu, Hui Xiong   +8 more
doaj   +1 more source

Multilevel analysis of nuclear dynamics in lamin perturbed fibroblasts [PDF]

, 2010
The nuclear lamina provides structural support to the nucleus and has a central role in defining nuclear organization. Defects in its filamentous constituents, the lamins, lead to a class of diseases collectively referred to as laminopathies.
Broers, Jos, De Vos, Winnok, Hoebe, Ron A, Houben, Frederik, Joss, Greg, Manders, Erik, Ramaekers, Frans, Van Oostveldt, Patric   +7 more
core  

Aging of Hutchinson-Gilford progeria syndrome fibroblasts is characterised by hyperproliferation and increased apoptosis [PDF]

, 2004
Hutchinson-Gilford progeria syndrome is a rare genetic disorder that mimics certain aspects of aging prematurely. Recent work has revealed that mutations in the lamin A gene are a cause of the disease.
Bridger, JM, Kill, IR
core   +1 more source