Results 51 to 60 of about 7,812 (251)

Heregulin‐induced epigenetic regulation of the utrophin‐A promoter [PDF]

FEBS Letters, 2007
Utrophin is the autosomal homolog of dystrophin, the product of the Duchenne's muscular dystrophy (DMD) locus. Utrophin is of therapeutic interest since its over‐expression can compensate dystrophin's absence. Utrophin is enriched at neuromuscular junctions due to heregulin‐mediated utrophin‐A promoter activation.
Utpal Basu, Olga Lozynska, Claus J. Jensen, Morten Frödin, Thomas O.B. Krag, Thomas O.B. Krag, Santhosh M. Baby, Tejvir S. Khurana, Mads Gyrd-Hansen   +8 more
openaire   +5 more sources

Marginal level dystrophin expression improves clinical outcome in a strain of dystrophin/utrophin double knockout mice. [PDF]

PLoS ONE, 2010
Inactivation of all utrophin isoforms in dystrophin-deficient mdx mice results in a strain of utrophin knockout mdx (uko/mdx) mice. Uko/mdx mice display severe clinical symptoms and die prematurely as in Duchenne muscular dystrophy (DMD) patients.
Dejia Li, Yongping Yue, Dongsheng Duan
doaj   +1 more source

Matricellular Protein CCN5 Gene Transfer Ameliorates Cardiac and Skeletal Dysfunction in mdx/utrn (±) Haploinsufficient Mice by Reducing Fibrosis and Upregulating Utrophin Expression

Frontiers in Cardiovascular Medicine, 2022
Duchenne muscular dystrophy (DMD) is a genetic disorder characterized by progressive muscle degeneration due to dystrophin gene mutations. Patients with DMD initially experience muscle weakness in their limbs during adolescence.
Min Ho Song, Jimeen Yoo, Jae Gyun Oh, Hyun Kook, Woo Jin Park, Dongtak Jeong, Dongtak Jeong   +6 more
doaj   +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

Correlation of Utrophin Levels with the Dystrophin Protein Complex and Muscle Fibre Regeneration in Duchenne and Becker Muscular Dystrophy Muscle Biopsies.

PLoS ONE, 2016
Duchenne muscular dystrophy is a severe and currently incurable progressive neuromuscular condition, caused by mutations in the DMD gene that result in the inability to produce dystrophin. Lack of dystrophin leads to loss of muscle fibres and a reduction
Narinder Janghra, Jennifer E Morgan, Caroline A Sewry, Francis X Wilson, Kay E Davies, Francesco Muntoni, Jonathon Tinsley   +6 more
doaj   +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

Preventing phosphorylation of dystroglycan ameliorates the dystrophic phenotype in mdx mouse [PDF]

, 2012
Loss of dystrophin protein due to mutations in the DMD gene causes Duchenne muscular dystrophy. Dystrophin loss also leads to the loss of the dystrophin glycoprotein complex (DGC) from the sarcolemma which contributes to the dystrophic phenotype ...
Andrew Mitchell, Andrä, Assereto, Batchelor, Bonuccelli, Bonuccelli, Chris J. Moore, Clerk, Cluchague, Cox, Deconinck, Dominic J. Wells, Ervasti, Ervasti, Ervasti, Foster, Gaynor Miller, Gazzerro, Goyenvalle, Grady, Greenberg, Gundesli, Hoyte, Hu, Ilsley, James, Judge, Karpati, Konieczny, Lipscomb, Matsumura, McArthur, Miller, Moore, Morgan, Nguyen, Otte, Pereboev, Pichavant, Pulkkinen, Rebecca Terry, Rezniczek, Robert Piggott, Schröder, Sharp, Smith, Sotgia, Sotgia, Steve J. Winder, T. Neil Dear, Thompson, Thompson, Tinsley, Tinsley, Tracy La Riviere, Winder   +55 more
core   +2 more sources

Post-natal induction of PGC-1α protects against severe muscle dystrophy independently of utrophin [PDF]

, 2014
Background: Duchenne muscle dystrophy (DMD) afflicts 1 million boys in the US and has few effective treatments. Constitutive transgenic expression of the transcriptional coactivator peroxisome proliferator-activated receptor gamma coactivator (PGC)-1α ...
Arany, Zolt, Chan, Mun Chun, Farrell, Caitlin, Patten, Ian S, Raghuram, Srilatha, Rowe, Glenn C   +5 more
core   +1 more source

The crystal structures of dystrophin and utrophin spectrin repeats: implications for domain boundaries.

PLoS ONE, 2012
Dystrophin and utrophin link the F-actin cytoskeleton to the cell membrane via an associated glycoprotein complex. This functionality results from their domain organization having an N-terminal actin-binding domain followed by multiple spectrin-repeat ...
Muralidharan Muthu, Kylie A Richardson, Andrew J Sutherland-Smith   +2 more
doaj   +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