Results 51 to 60 of about 57,573 (289)

Increased levels of interleukin-6 exacerbate the dystrophic phenotype in mdx mice [PDF]

, 2015
Duchenne muscular dystrophy (DMD) is characterized by progressive lethal muscle degeneration and chronic inflammatory response. The mdx mouse strain has served as the animal model for human DMD. However, while DMD patients undergo extensive necrosis, the
Berardinelli, Maria Grazia, Camilli, Carlotta, Catizone, Angela, De Benedetti, Fabrizio, Forcina, Laura, Musarò, Antonio, Nicoletti, Carmine, Pelosi, Laura, Rizzuto, Emanuele, Spelta, Elisa, Testa, Erika   +10 more
core   +2 more sources

ETV2 Mediated Differentiation of Human Pluripotent Stem Cells Results in Functional Endothelial Cells for Engineering Advanced Vascularized Microphysiological Models

Advanced Healthcare Materials, EarlyView.
A robust method to generate functional human iPSC‐derived endothelial cells using inducible ETV2 expression. These cells self‐organize into stable, lumenized microvascular networks within microfluidic chips, surpassing conventional differentiation methods.
Shun Zhang, Zhengpeng Wan, Lei Wang, Caihong Wu, Junkai Zhang, Sarah Spitz, Xun Wang, Marie A. Floryan, Mark F. Coughlin, Francesca M. Pramotton, Liling Xu, Ron Weiss, Roger D. Kamm   +12 more
wiley   +1 more source

Desmin gene expression is not ubiquitous in all upper airway myofibers and the pattern differs between healthy and sleep apnea subjects

European Journal of Medical Research
Background Desmin is a major cytoskeletal protein considered ubiquitous in mature muscle fibers. However, we earlier reported that a subgroup of muscle fibers in the soft palate of healthy subjects and obstructive sleep apnea patients (OSA) lacked ...
Per Stål, Hanna Nord, Jonas von Hofsten, Thorbjörn Holmlund, Farhan Shah   +4 more
doaj   +1 more source

Thioflavin T in-gel staining for ex vivo analysis of cardiac amyloid

Frontiers in Molecular Biosciences
There are limited options to quantify and characterize amyloid species from biological samples in a simple manner. Thioflavin T (ThT) has been used for decades to stain amyloid fibrils, but to our knowledge, we were the first to use it in-gel. Thioflavin
Joseph Oldam, Irina Tchernyshyov, Jennifer Van Eyk, Juan Troncoso, Charles G. Glabe, Giulio Agnetti, Giulio Agnetti   +6 more
doaj   +1 more source

Developmental Alterations in Heart Biomechanics and Skeletal Muscle Function in Desmin Mutants Suggest an Early Pathological Root for Desminopathies

Cell Reports, 2015
Desminopathies belong to a family of muscle disorders called myofibrillar myopathies that are caused by Desmin mutations and lead to protein aggregates in muscle fibers.
Caroline Ramspacher, Emily Steed, Francesco Boselli, Rita Ferreira, Nathalie Faggianelli, Stéphane Roth, Coralie Spiegelhalter, Nadia Messaddeq, Le Trinh, Michael Liebling, Nikhil Chacko, Federico Tessadori, Jeroen Bakkers, Jocelyn Laporte, Karim Hnia, Julien Vermot   +15 more
doaj   +1 more source

Hepatoma‐Derived Growth Factor Coordinates STAT3 Pathway and Exosome‐Mediated Intrahepatic Crosstalk to Control Hepatic Steatosis and MASLD

Advanced Science, EarlyView.
Hepatic HDGF as a key mediator in coordinating hepatic steatosis and intrahepatic crosstalk in MASLD. Activation of HDGF facilitates its interaction with both STAT3 and S6K1, driving the S6K1‐dependent STAT3 phosphorylation and subsequently enhancing hepatic lipogenesis.
Jian Wen, Dong Ding, Zengpeng Zheng, Xufeng Chen, Wenjing Li, Jiaxin Shen, Zhiwei Huang, Peng Tan, Junjie Bai, Xia Fang, Baofang Xing, Puyuan Hu, Linghao Xu, Haokai Yu, Yixi Wang, Zongzhe Jiang, Yang Long, Tiejun Zhou, Mingxin Ye, Yu Jiang, Aoyuan Cui, Hong Li, Qiurong Ding, Yong Xu, Yu Li, Weitong Su, Feng Shen, Chenlin Gao, Wenguang Fu   +28 more
wiley   +1 more source

Nebulette knockout mice have normal cardiac function, but show Z-line widening and up-regulation of cardiac stress markers [PDF]

, 2015
Aims: Nebulette is a 109 kDa modular protein localized in the sarcomeric Z-line of the heart. In vitro studies have suggested a role of nebulette in stabilizing the thin filament, and missense mutations in the nebulette gene were recently shown to be ...
Bang, Marie-Louise, Carullo, Pierluigi, Chen, Ju, Dalton, Nancy D., Gu, Yusu, Li, Xiaodong, Liang, Xingqun, Mastrototaro, Giuseppina, Peterson, Kirk L., Piroddi, Nicoletta, Poggesi, Corrado, Sheikh, Farah, Tesi, Chiara   +12 more
core   +2 more sources

Targeted Anti‐IL‐1 Immunomodulatory Therapy in Pediatric Onset PPP1R13L‐Related Arrhythmogenic Cardiomyopathy

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Autosomal recessive loss‐of‐function variants in PPP1R13L cause an ultra‐rare cardiocutaneous syndrome characterized by rapidly progressive arrhythmogenic cardiomyopathy (ACM). PPP1R13L encodes iASPP, which has two potentially overlapping mechanisms driving ACM as both a regulator of NFκB‐mediated inflammation and a binding partner within the ...
Aaron Renberg, Savannah Coppersmith, Owen Merritt, Amer Heider, Adam Helms, Thomas Michniacki, Jack Luxford, Sarah Josephi‐Taylor, Philip Roberts, Joshua Meisner   +9 more
wiley   +1 more source

Changes in cell shape and desmin intermediate filament distribution are associated with down-regulation of desmin expression in C2C12 myoblasts grown in the absence of extracellular Ca2+

Brazilian Journal of Medical and Biological Research, 2005
Desmin is the main intermediate filament (IF) protein of muscle cells. In skeletal muscle, desmin IFs form a scaffold that interconnects the entire contractile apparatus with the subsarcolemmal cytoskeleton and cytoplasmic organelles.
C.S. Mermelstein, L.M. Amaral, M.I.L. Rebello, J.S.N. Reis, R. Borojevic, M.L. Costa   +5 more
doaj   +1 more source

Small heat-shock proteins: important players in regulating cellular proteostasis [PDF]

, 2015
Small heat-shock proteins (sHsps) are a diverse family of intra-cellular molecular chaperone proteins that play a critical role in mitigating and preventing protein aggregation under stress conditions such as elevated temperature, oxidation and infection.
Carver, John A., Ecroyd, Heath, Meehan, Sarah, Treweek, Teresa M.   +3 more
core   +1 more source