Results 11 to 20 of about 7,758 (250)

Utrophin Compensates dystrophin Loss during Mouse Spermatogenesis [PDF]

Scientific Reports, 2017
Duchenne muscular dystrophy (DMD) is an X-linked genetic disorder resulting from mutations in the dystrophin gene. The mdx/utrn −/− mouse, lacking in both dystrophin and its autosomal homologue utrophin, is commonly used to model the clinical symptoms of
Hung-Chih Chen, Yu-Feng Chin, David J. Lundy, Chung-Tiang Liang, Ya-Hui Chi, Paolin Kuo, Patrick C. H. Hsieh   +6 more
doaj   +5 more sources

Association of Aciculin with Dystrophin and Utrophin [PDF]

Journal of Biological Chemistry, 1995
Aciculin is a recently identified 60-kDa cytoskeletal protein, highly homologous to the glycolytic enzyme phosphoglucomutase type 1, (Belkin, A. M., Klimanskaya, I. V., Lukashev, M. E., Lilley, K., Critchley, D., and Koteliansky, V. E. (1994) J. Cell Sci. 107, 159-173).
Alexey M. Belkin, Keith Burridge
openalex   +5 more sources

Utrophin: A Structural and Functional Comparison to Dystrophin [PDF]

Brain Pathology, 1996
Utrophin is an autosomally‐encoded homologue of dystrophin, the protein product of the Duchenne muscular dystrophy (DMD) gene. Although, Utrophin is very similar in sequence to dystrophin and possesses many of the protein‐binding properties ascribed to dystrophin, both proteins are expressed in an apparently reciprocal manner and may be coordinately ...
Derek J. Blake, Jonathon M. Tinsley, Kay E. Davies   +2 more
openalex   +4 more sources

Disruption of the utrophin‒actin interaction by monoclonal antibodies and prediction of an actin-binding surface of utrophin [PDF]

Biochemical Journal, 1999
Monoclonal antibody (mAb) binding sites in the N-terminal actin-binding domain of utrophin have been identified using phage-displayed peptide libraries, and the mAbs have been used to probe functional regions of utrophin involved in actin binding. mAbs were characterized for their ability to interact with the utrophin actin-binding domain and to affect
Glenn E. Morris, Nguyen thi Mân, Nguyen thi Ngoc HUYEN, Alexander PEREBOEV, John Kendrick‐Jones, Steven J. Winder   +5 more
openalex   +3 more sources

Sticky utrophin messages

The Journal of Cell Biology, 2001
![Graphic][1] Utrophin mRNAs bind to actin. On [page 1173][2], Gramolini et al. report that utrophin mRNA is immobilized by binding to an actin-dependent structure. Manipulation of this system may be important for the therapy of Duchenne muscular dystrophy (DMD).
William A. Wells
openalex   +4 more sources

Functional substitution by TAT-utrophin in dystrophin-deficient mice. [PDF]

PLoS Medicine, 2009
The loss of dystrophin compromises muscle cell membrane stability and causes Duchenne muscular dystrophy and/or various forms of cardiomyopathy. Increased expression of the dystrophin homolog utrophin by gene delivery or pharmacologic up-regulation has ...
Kevin J Sonnemann, Hanke Heun-Johnson, Amy J Turner, Kristen A Baltgalvis, Dawn A Lowe, James M Ervasti   +5 more
doaj   +3 more sources

Daily treatment with SMTC1100, a novel small molecule utrophin upregulator, dramatically reduces the dystrophic symptoms in the mdx mouse.

PLoS ONE, 2011
BackgroundDuchenne muscular dystrophy (DMD) is a lethal, progressive muscle wasting disease caused by a loss of sarcolemmal bound dystrophin, which results in the death of the muscle fibers leading to the gradual depletion of skeletal muscle.
Jonathon M Tinsley, Rebecca J Fairclough, Richard Storer, Fraser J Wilkes, Allyson C Potter, Sarah E Squire, Dave S Powell, Anna Cozzoli, Roberta F Capogrosso, Adam Lambert, Francis X Wilson, Stephen P Wren, Annamaria De Luca, Kay E Davies   +13 more
doaj   +2 more sources

Fatigue Resistance and Mitochondrial Adaptations to Isometric Interval Training in Dystrophin-Deficient Muscle: Role of Contractile Load. [PDF]

FASEB J
High‐load, but not low‐load, isometric interval training (IT) increased mitochondrial content and muscle fatigue resistance in mdx52 mice, a model for Duchenne muscular dystrophy. High‐load isometric IT restored citrate synthase activity, peroxisome proliferator‐activated receptor γ coactivator 1 alpha (PGC‐1α) expression, and mitochondrial complex II ...
Yamauchi N, Ashida Y, Naito A, Tokuda N, Niibori A, Motohashi N, Aoki Y, Yamada T.   +7 more
europepmc   +2 more sources

Mitochondrial stress in advanced fibrosis and cirrhosis associated with chronic hepatitis B, chronic hepatitis C, or nonalcoholic steatohepatitis

Hepatology, EarlyView., 2022
Adaptive mitochondrial mechanisms allow mitochondrial resilience and prevent the worsening of fibrosis, while deregulation of these mechanisms promotes the progression from no/minimal‐mild (F0‐F2) fibrosis to advanced fibrosis and cirrhosis (F3‐F4). Abstract Background and Aims Hepatitis B virus (HBV) infection causes oxidative stress (OS) and alters ...
Dimitri Loureiro, Issam Tout, Stéphanie Narguet, Cheikh Mohamed Bed, Morgane Roinard, Ahmad Sleiman, Nathalie Boyer, Nathalie Pons‐Kerjean, Corinne Castelnau, Nathalie Giuly, Dorothy Tonui, Vassili Soumelis, Jamel El Benna, Patrick Soussan, Richard Moreau, Valérie Paradis, Abdellah Mansouri, Tarik Asselah   +17 more
wiley   +1 more source

Sertoli Cells Improve Myogenic Differentiation, Reduce Fibrogenic Markers, and Induce Utrophin Expression in Human DMD Myoblasts

Biomolecules, 2021
Duchenne muscular dystrophy (DMD) is an X-linked disease caused by mutations in DMD gene translating in lack of functional dystrophin and resulting in susceptibility of myofibers to rupture during contraction.
Laura Salvadori, Sara Chiappalupi, Iva Arato, Francesca Mancuso, Mario Calvitti, Maria Cristina Marchetti, Francesca Riuzzi, Riccardo Calafiore, Giovanni Luca, Guglielmo Sorci   +9 more
doaj   +1 more source