Results 71 to 80 of about 38,496 (259)

Altered muscle niche contributes to myogenic deficit in the D2-mdx model of severe DMD

Cell Death Discovery, 2023
Lack of dystrophin expression is the underlying genetic basis for Duchenne muscular dystrophy (DMD). However, disease severity varies between patients, based on specific genetic modifiers. D2-mdx is a model for severe DMD that exhibits exacerbated muscle
Davi A. G. Mázala, Ravi Hindupur, Young Jae Moon, Fatima Shaikh, Iteoluwakishi H. Gamu, Dhruv Alladi, Georgiana Panci, Michèle Weiss-Gayet, Bénédicte Chazaud, Terence A. Partridge, James S. Novak, Jyoti K. Jaiswal   +11 more
doaj   +1 more source

Normalizing the Taylor expansion of non-deterministic {\lambda}-terms, via parallel reduction of resource vectors

, 2019
It has been known since Ehrhard and Regnier's seminal work on the Taylor expansion of $\lambda$-terms that this operation commutes with normalization: the expansion of a $\lambda$-term is always normalizable and its normal form is the expansion of the B\"
Vaux, Lionel
core   +3 more sources

Restoration of pharyngeal dilator muscle force in dystrophin-deficient (mdx) mice following co-treatment with neutralizing interleukin-6 receptor antibodies and urocortin-2 [PDF]

, 2017
New Findings: What is the central question of this study? We previously reported impaired upper airway dilator muscle function in the mdx mouse model of Duchenne muscular dystrophy (DMD).
Brannock, Molly, Burns, David P., Canavan, Leonie, Edge, Deirdre, Murphy, Kevin H., O'Halloran, Ken D., O'Malley, Dervla, Rowland, Jane   +7 more
core   +1 more source

On an Aggregation Theory for Indicators Expressing Behaviors of Complex Systems With an Application to Sustainability

Sustainable Development, EarlyView.
ABSTRACT Certain attributes of large‐scale complex systems are often expressed through sets of indicators. For example, the sustainability of an entity, be it a nation, a city, an energy system, a corporation etc., can be effectively represented by indicators and corresponding data series.
Vassilis S. Kouikoglou, Yannis A. Phillis   +1 more
wiley   +1 more source

Multi-omics analysis of sarcospan overexpression in mdx skeletal muscle reveals compensatory remodeling of cytoskeleton-matrix interactions that promote mechanotransduction pathways

Skeletal Muscle, 2023
Background The dystrophin-glycoprotein complex (DGC) is a critical adhesion complex of the muscle cell membrane, providing a mechanical link between the extracellular matrix (ECM) and the cortical cytoskeleton that stabilizes the sarcolemma during ...
Jackie L. McCourt, Kristen M. Stearns-Reider, Hafsa Mamsa, Pranav Kannan, Mohammad Hossein Afsharinia, Cynthia Shu, Elizabeth M. Gibbs, Kara M. Shin, Yerbol Z. Kurmangaliyev, Lauren R. Schmitt, Kirk C. Hansen, Rachelle H. Crosbie   +11 more
doaj   +1 more source

An ex vivo gene therapy approach to treat muscular dystrophy using inducible pluripotent stem cells. [PDF]

, 2013
Duchenne muscular dystrophy is a progressive and incurable neuromuscular disease caused by genetic and biochemical defects of the dystrophin-glycoprotein complex.
Borges, Luciene, Chamberlain, Jeffrey S, Darabi, Radbod, Ervasti, James M, Filareto, Antonio, Iacovino, Michelina, Kyba, Michael, Mayerhofer, Timothy, McIvor, R Scott, Parker, Sarah, Perlingeiro, Rita CR, Schaaf, Tory   +11 more
core   +2 more sources

Nature Inspired Delivery Vehicles for CRISPR‐Based Genome Editing

Small, EarlyView.
The review highlights nature‐inspired nanocarriers for CRISPR delivery, emphasizing viral vectors, extracellular vesicles, liposomes, and lipid nanoparticles. It discusses their roles in improving specificity, minimizing immunogenicity, and overcoming barriers in genome editing. Recent advancements, challenges, and therapeutic applications are explored,
Elizabeth Maria Clarissa, Mamata Karmacharya, Hyunmin Choi, Sumit Kumar, Yoon‐Kyoung Cho   +4 more
wiley   +1 more source

Framework for X‐ray mirror surface shape fitting

Journal of Synchrotron Radiation, EarlyView.
The closed‐form expressions for elliptic cylinders, hyperbolic cylinders, ellipsoids, hyperboloids, and diaboloids used for X‐ray mirror surface shapes are summarized. Additionally, a four‐layer framework to fit the measured surface shape data in slope or height for convenient X‐ray mirror surface characterization is proposed.For accurate ...
Lei Huang, Ruochen Xu, Tianyi Wang, Jumpei Yamada, Joseph Dvorak, Corey Austin, Albert Van Eeckhout, Josep Nicolàs Roman, Kenneth Goldberg, Mourad Idir   +9 more
wiley   +1 more source

Transgenic overexpression of γ-cytoplasmic actin protects against eccentric contraction-induced force loss in mdx mice

Skeletal Muscle, 2011
Background γ-cytoplasmic (γ-cyto) actin levels are elevated in dystrophin-deficient mdx mouse skeletal muscle. The purpose of this study was to determine whether further elevation of γ-cyto actin levels improve or exacerbate the dystrophic phenotype of ...
Baltgalvis Kristen A, Jaeger Michele A, Fitzsimons Daniel P, Thayer Stanley A, Lowe Dawn A, Ervasti James M   +5 more
doaj   +1 more source

The unconditioned fear response in dystrophin-deficient mice is associated with adrenal and vascular function

Scientific Reports, 2023
Loss of function mutations in the gene encoding dystrophin elicits a hypersensitive fear response in mice and humans. In the dystrophin-deficient mdx mouse, this behaviour is partially protected by oestrogen, but the mechanistic basis for this protection
Angus Lindsay, Aaron P. Russell
doaj   +1 more source