Results 51 to 60 of about 7,173 (197)

Microutrophin expression in dystrophic mice displays myofiber type differences in therapeutic effects.

PLoS Genetics, 2020
Gene therapy approaches for DMD using recombinant adeno-associated viral (rAAV) vectors to deliver miniaturized (or micro) dystrophin genes to striated muscles have shown significant progress.
Glen B Banks, Jeffrey S Chamberlain, Guy L Odom   +2 more
doaj   +1 more source

Steric regulation of tandem calponin homology domain actin-binding affinity. [PDF]

, 2019
Tandem calponin homology (CH1-CH2) domains are common actin-binding domains in proteins that interact with and organize the actin cytoskeleton. Despite regions of high sequence similarity, CH1-CH2 domains can have remarkably different actin-binding ...
Bausch, Andreas, Belardi, Brian, Fletcher, Daniel A, Harris, Andrew R, Jreij, Pamela, Shams, Hengameh, Wei, Kathy   +6 more
core   +1 more source

Polyplex Nanomicelle‐Mediated Pgc‐1α4 mRNA Delivery Via Hydrodynamic Limb Vein Injection Enhances Damage Resistance in Duchenne Muscular Dystrophy Mice

Advanced Science, EarlyView.
This study presents a novel approach for treating Duchenne muscular dystrophy using mRNA encoding PGC‐1α4. Nanomicelle‐delivered Pgc‐1α4 mRNA enhances muscle damage resistance and mitochondrial activity in dystrophic muscles. This study demonstrates the potential of mRNA therapy for neuromuscular diseases like Duchenne muscular dystrophy and highlights
Xuan Du, Hideyuki Nakanishi, Takashi Yamada, Yooksil Sin, Katsura Minegishi, Norio Motohashi, Yoshitsugu Aoki, Keiji Itaka   +7 more
wiley   +1 more source

Dystrophin glycoprotein complex dysfunction:a regulatory link between muscular dystrophy and cancer cachexia [PDF]

, 2005
SummaryCachexia contributes to nearly a third of all cancer deaths, yet the mechanisms underlying skeletal muscle wasting in this syndrome remain poorly defined.
Acharyya, Swarnali, Burghes, Arthur H M, Butchbach, Matthew E R, Carathers, Micheal, Fearon, Kenneth C H, Guttridge, Denis C, Hollingsworth, Michael A, Muscarella, Peter, Rafael-Fortney, Jill A, Ringel, Matthew D, Sahenk, Zarife, Saji, Motoyasu, Skipworth, Richard J E, Wang, Huating   +13 more
core   +1 more source

The Heterotaxy Gene CCDC11 Is Important for Cytokinesis via RhoA Regulation

Cytoskeleton, EarlyView.
ABSTRACT Mutations in CCDC11 (cfap53) have been identified in multiple patients with heterotaxy (Htx), a disorder of left–right (LR) patterning of the internal organs. In Xenopus, depletion of Ccdc11 causes defects in LR patterning, recapitulating the patient phenotype. Upon Ccdc11 depletion, monociliated cells of the Left–Right Organizer (LRO) exhibit
Saurabh S. Kulkarni, Rachel E. Stephenson, Sarah Amalraj, Angelo Arrigo, Ewelina Betleja, James J. Moresco, John R. Yates III, Moe R. Mahjoub, Ann L. Miller, Mustafa K. Khokha   +9 more
wiley   +1 more source

An analysis of contractile and protrusive cell behaviors at the superficial surface of the zebrafish neural plate

Developmental Dynamics, EarlyView.
Abstract Background The forces underlying convergence and internalization of the teleost neural plate remain unknown. To help understand this morphogenesis, we analyzed collective and individual cell behaviors at the superficial surface of the neural plate as internalization begins to form the neural keel in the hindbrain region of the zebrafish embryo.
Claudio Araya, Raegan Boekemeyer, Francesca Farlie, Lauren Moon, Freshta Darwish, Chris Rookyard, Leanne Allison, Gema Vizcay‐Barrena, Roland Fleck, Millaray Aranda, Masa Tada, Jonathan D. W. Clarke   +11 more
wiley   +1 more source

Modulation of PGC-1α activity as a treatment for metabolic and muscle-related diseases [PDF]

, 2014
Physical inactivity is a predisposing factor for various disease states including obesity, cardiovascular disease, as well as for certain types of cancer. Regular endurance exercise mediates several beneficial effects such as increased energy expenditure
Handschin, Christoph, Svensson, Kristoffer   +1 more
core   +1 more source

Genome Editing-Mediated Utrophin Upregulation in Duchenne Muscular Dystrophy Stem Cells

Molecular Therapy: Nucleic Acids, 2020
Utrophin upregulation is considered a promising therapeutic strategy for Duchenne muscular dystrophy (DMD). A number of microRNAs (miRNAs) post-transcriptionally regulate utrophin expression by binding their cognate sites in the 3′ UTR.
Kasturi Sengupta, Manoj K. Mishra, Emanuele Loro, Melissa J. Spencer, April D. Pyle, Tejvir S. Khurana   +5 more
doaj  

Endogenous bioluminescent reporters reveal a sustained increase in utrophin gene expression upon EZH2 and ERK1/2 inhibition

Communications Biology, 2023
With the long-term goal of finding therapeutic options for Duchenne muscular dystrophy, a mouse model enabling simultaneous visualisation of Dmd and Utrn is described and inhibitors of PRC2 and ERK1/2 identified to increase utrophin expression.
Hannah J. Gleneadie, Beatriz Fernandez-Ruiz, Alessandro Sardini, Mathew Van de Pette, Andrew Dimond, Rab K. Prinjha, James McGinty, Paul M. W. French, Hakan Bagci, Matthias Merkenschlager, Amanda G. Fisher   +10 more
doaj   +1 more source

Molecular interactions of the intermediate filament protein synemin with both dystrophin and utrophin in muscle cells [PDF]

, 2006
Synemin is a very unique, large intermediate filament (IF) protein present in all types of muscle cells. It forms heteropolymeric intermediate filaments (IFs) with the major IF proteins desmin and/or vimentin.
Bhosle, Rahul Chandrakant
core   +4 more sources