Results 91 to 100 of about 1,104,554 (244)

Circadian Genes as Exploratory Biomarkers in DMD: Results From Both the mdx Mouse Model and Patients

Frontiers in Physiology, 2021
Duchenne muscular dystrophy (DMD) is a rare genetic disease due to dystrophin gene mutations which cause progressive weakness and muscle wasting. Circadian rhythm coordinates biological processes with the 24-h cycle and it plays a key role in maintaining
Rachele Rossi, Rachele Rossi, Maria Sofia Falzarano, Hana Osman, Hana Osman, Annarita Armaroli, Chiara Scotton, Paola Mantuano, Brigida Boccanegra, Ornella Cappellari, Elena Schwartz, Anton Yuryev, Eugenio Mercuri, Enrico Bertini, Adele D’Amico, Marina Mora, Camilla Johansson, Cristina Al-Khalili Szigyarto, Cristina Al-Khalili Szigyarto, Annamaria De Luca, Alessandra Ferlini, Alessandra Ferlini   +21 more
doaj   +1 more source

Volitional exercise elicits physiological and molecular improvements in the severe D2.mdx mouse model of Duchenne muscular dystrophy

The Journal of Physiology, EarlyView.
Abstract figure legend This study investigated the effects of volitional exercise on muscle health in the more severe D2.mdx model of Duchenne muscular dystrophy (DMD). We showed that 8–10 weeks of a relatively high volume of voluntary wheel running (VWR) in D2.mdx animals augmented select muscle mass and normalized ex vivo muscle force compared to ...
Stephanie R. Mattina, Sean Y. Ng, Andrew I. Mikhail, Derek W. Stouth, Cora E. Jornacion, Irena A. Rebalka, Thomas J. Hawke, Vladimir Ljubicic   +7 more
wiley   +1 more source

In Vivo Gene Editing of Muscle Stem Cells with Adeno-Associated Viral Vectors in a Mouse Model of Duchenne Muscular Dystrophy

Molecular Therapy: Methods & Clinical Development, 2020
Delivery of therapeutic transgenes with adeno-associated viral (AAV) vectors for treatment of myopathies has yielded encouraging results in animal models and early clinical studies.
Jennifer B. Kwon, Adarsh R. Ettyreddy, Ashish Vankara, Joel D. Bohning, Garth Devlin, Stephen D. Hauschka, Aravind Asokan, Charles A. Gersbach   +7 more
doaj   +1 more source

Cathelicidin‐related antimicrobial peptide mediates skeletal muscle degeneration caused by injury and Duchenne muscular dystrophy in mice

Journal of Cachexia, Sarcopenia and Muscle, 2022
Background Cathelicidin, an antimicrobial peptide, plays a key role in regulating bacterial killing and innate immunity; however, its role in skeletal muscle function is unknown.
Moon‐Chang Choi, Jiwon Jo, Myeongjin Lee, Jonggwan Park, Tso‐Pang Yao, Yoonkyung Park   +5 more
doaj   +1 more source

Targeting a therapeutic LIF transgene to muscle via the immune system ameliorates muscular dystrophy. [PDF]

, 2019
Many potentially therapeutic molecules have been identified for treating Duchenne muscular dystrophy. However, targeting those molecules only to sites of active pathology is an obstacle to their clinical use.
Bertoni, Carmen, Flores, Ivan, Ramos, Julian, Tidball, James G, Wang, Ying, Wehling-Henricks, Michelle, Welc, Steven S   +6 more
core  

Review: Dystroglycan in the Nervous System [PDF]

, 2007
Dystroglycan is part of a large complex of proteins, the dystrophin-glycoprotein complex, which has been implicated in the pathogenesis of muscular dystrophies for a long time.
Matthias Samwald
core   +2 more sources

The mitochondrial‐targeted antioxidant SkQ1 prevents skeletal muscle mitochondrial‐apoptotic but not necroptotic signalling during ovarian cancer

The Journal of Physiology, EarlyView.
Abstract figure legend An evaluation of the degree to which mitochondrial hydrogen peroxide emission (mH2O2)‐mediated apoptotic and necroptotic signalling contributes to skeletal muscle atrophy in an orthotopic epithelial ovarian cancer (EOC) model. To determine whether attenuating mH2O2 could prevent regulated cell death signalling and mitigate muscle
Shahrzad Khajehzadehshoushtar, Luca J. Delfinis, Fasih A. Rahman, Madison C. Garibotti, Shivam Gandhi, Aditya N. Brahmbhatt, Brooke A. Morris, Bianca Garlisi, Sylvia Lauks, Caroline Aitken, Leslie Ogilvie, Zhu‐Xu Zhang, Jeremy A. Simpson, Joe Quadrilatero, Jim Petrik, Christopher G. R. Perry   +15 more
wiley   +1 more source

Measuring neuromuscular junction functionality [PDF]

, 2017
Neuromuscular junction (NMJ) functionality plays a pivotal role when studying diseases in which the communication between motor neuron and muscle is impaired, such as aging and amyotrophic lateral sclerosis (ALS).
Del Prete, Zaccaria, Musaro, Antonio, Nicoletti, Carmine, Pisu, Simona, Rizzuto, Emanuele   +4 more
core   +1 more source

Myostatin and activin blockade by engineered follistatin results in hypertrophy and improves dystrophic pathology in mdx mouse more than myostatin blockade alone

Skeletal Muscle, 2018
BackgroundMyostatin antagonists are being developed as therapies for Duchenne muscular dystrophy due to their strong hypertrophic effects on skeletal muscle. Engineered follistatin has the potential to combine the hypertrophy of myostatin antagonism with
A. Iskenderian, Nan Liu, Qingwei Deng, Yan Huang, Chuan Shen, Kathleen Palmieri, R. Crooker, Dianna M Lundberg, N. Kastrapeli, B. Pescatore, A. Romashko, John Dumas, Robert Comeau, Angela W. Norton, Jing Pan, H. Rong, K. Derakhchan, D. Ehmann   +17 more
semanticscholar   +1 more source

Evidence for ACTN3 as a genetic modifier of Duchenne muscular dystrophy [PDF]

, 2017
Duchenne muscular dystrophy (DMD) is characterized by muscle degeneration and progressive weakness. There is considerable inter-patient variability in disease onset and progression, which can confound the results of clinical trials.
, Bello, Luca, Gordish-Dressman, Heather, Head, Stewart I., Hoffman, Eric P., Hogarth, Marshall W., Houweling, Peter J., North, Kathryn N., Pegoraro, Elena, Thomas, Kristen C.   +9 more
core   +2 more sources