Results 41 to 50 of about 38,378 (273)

Oxygen Defects and Instability in Very Thin a‐IGZO TFTs

Advanced Electronic Materials
Amorphous oxide semiconductor (AOS) thin‐film transistors (TFT) have gained significant attention for their potential in capacitor‐free next‐generation memory applications.
Hanjun Cho, Masatake Tsuji, Shigenori Ueda, Junghwan Kim, Hideo Hosono   +4 more
doaj   +1 more source

Fibroadipogenic progenitors mediate the ability of HDAC inhibitors to promote regeneration in dystrophic muscles of young, but not old Mdx mice

EMBO Molecular Medicine, 2013
HDAC inhibitors (HDACi) exert beneficial effects in mdx mice, by promoting endogenous regeneration; however, the cellular determinants of HDACi activity on dystrophic muscles have not been determined.
Chiara Mozzetta, Silvia Consalvi, Valentina Saccone, Matthew Tierney, Adamo Diamantini, Kathryn J. Mitchell, Giovanna Marazzi, Giovanna Borsellino, Luca Battistini, David Sassoon, Alessandra Sacco, Pier Lorenzo Puri   +11 more
doaj   +1 more source

Taurine and Methylprednisolone Administration at Close Proximity to the Onset of Muscle Degeneration Is Ineffective at Attenuating Force Loss in the Hind-Limb of 28 Days Mdx Mice

Sports, 2018
An increasing number of studies have shown supplementation with the amino acid taurine to have promise in ameliorating dystrophic symptoms in the mdx mouse model of Duchenne Muscular Dystrophy (DMD).
Robert G. Barker, Chris van der Poel, Deanna Horvath, Robyn M. Murphy   +3 more
doaj   +1 more source

Electride‐Induced Electronic Modulation of Ruthenium Catalyst for Highly Efficient Alkaline Hydrogen Evolution

Advanced Science, EarlyView.
Electrides offer unique opportunities as catalyst supports for hydrogen evolution reactions. This study presents an electride‐supported Ru/Nd2ScSi2 catalyst exhibiting outstanding hydrogen evolution reaction performance and excellent stability. Anionic Ru species on the electride surface facilitate water dissociation and optimize hydrogen adsorption ...
Zhiqi Wang, Jianan Su, Cheng Li, Yutong Gong, Hideo Hosono, Junjie Wang   +5 more
wiley   +1 more source

Dysfunctional muscle and liver glycogen metabolism in mdx dystrophic mice. [PDF]

PLoS ONE, 2014
Duchenne muscular dystrophy (DMD) is a severe, genetic muscle wasting disorder characterised by progressive muscle weakness. DMD is caused by mutations in the dystrophin (dmd) gene resulting in very low levels or a complete absence of the dystrophin ...
David I Stapleton, Xianzhong Lau, Marcelo Flores, Jennifer Trieu, Stefan M Gehrig, Annabel Chee, Timur Naim, Gordon S Lynch, René Koopman   +8 more
doaj   +1 more source

A nitric oxide synthase transgene ameliorates muscular dystrophy in mdx mice. [PDF]

, 2001
Dystrophin-deficient muscles experience large reductions in expression of nitric oxide synthase (NOS), which suggests that NO deficiency may influence the dystrophic pathology.
Spencer, MJ, Tidball, JG, Wehling, M
core  

Targeting RyR Activity Boosts Antisense Exon 44 and 45 Skipping in Human DMD Skeletal or Cardiac Muscle Culture Models. [PDF]

, 2019
Systemic delivery of antisense oligonucleotides (AO) for DMD exon skipping has proven effective for reframing DMD mRNA, rescuing dystrophin expression, and slowing disease progression in animal models.
Barthélémy, Florian, Douine, Emilie D, Hsu, Christopher, Marcantonio, Eugene E, Miceli, M Carrie, Nelson, Stanley F, Wang, Richard T   +6 more
core   +1 more source

Mutation type‐specific transcriptomic signatures and readthrough therapy rescue in SMC1A‐related developmental and epileptic encephalopathy

Epilepsia, EarlyView.
Abstract Objective This study was undertaken to investigate the molecular consequences of pathogenic variants in the SMC1A gene—particularly those associated with developmental and epileptic encephalopathy (DEE85)—and to evaluate the therapeutic potential of ataluren in restoring SMC1A function and mitigating disease‐related transcriptomic and genomic ...
Maddalena Di Nardo, Francesca Sardina, Maria M. Pallotta, Iñigo Marcos‐Alcalde, Paulino Gómez‐Puertas, Cinzia Rinaldo, Ian D. Krantz, Antonio Musio   +7 more
wiley   +1 more source

Asynchrony Related to Mechanical Ventilation Exacerbates Diaphragm Dysfunction in a Mouse Model of Duchenne Muscular Dystrophy

Muscle &Nerve, EarlyView.
ABSTRACT Introduction/Aims In dystrophic mice (mdx, a genetic homolog of Duchenne muscular dystrophy: DMD), previous studies showed that mechanical ventilation (MV) induces ventilator‐induced diaphragmatic dysfunction (VIDD). However, susceptibility to mechanical stress caused by asynchrony remains unknown.
Mohamad Yehya, Haikel Dridi, Eric Estève, Johan Moreau, Steven Reiken, Andrew R. Marks, Alain Lacampagne, Stefan Matecki   +7 more
wiley   +1 more source

Nifedipine treatment reduces resting calcium concentration, oxidative and apoptotic gene expression, and improves muscle function in dystrophic mdx mice. [PDF]

PLoS ONE, 2013
Duchenne Muscular Dystrophy (DMD) is a recessive X-linked genetic disease, caused by mutations in the gene encoding dystrophin. DMD is characterized in humans and in mdx mice by a severe and progressive destruction of muscle fibers, inflammation ...
Francisco Altamirano, Denisse Valladares, Carlos Henríquez-Olguín, Mariana Casas, Jose R López, Paul D Allen, Enrique Jaimovich   +6 more
doaj   +1 more source