Results 31 to 40 of about 18,188 (219)

Dystrophin Stabilizes α3- But Not α7-Containing Nicotinic Acetylcholine Receptor Subtypes at the Postsynaptic Apparatus in the Mouse Superior Cervical Ganglion

Neurobiology of Disease, 2002
The nicotinic acetylcholine receptor (nAChR) subtypes were characterized in the superior cervical ganglion (SCG) of wild-type and dystrophin-lacking mdx mice.
Arianna Del Signore, Cecilia Gotti, M.Egle De Stefano, Milena Moretti, Paola Paggi   +4 more
doaj   +1 more source

Osteopontin ablation ameliorates muscular dystrophy by shifting macrophages to a pro-regenerative phenotype. [PDF]

, 2016
In the degenerative disease Duchenne muscular dystrophy, inflammatory cells enter muscles in response to repetitive muscle damage. Immune factors are required for muscle regeneration, but chronic inflammation creates a profibrotic milieu that exacerbates
Barton, Elisabeth R, Capote, Joana, Kramerova, Irina, Martinez, Leonel, Miceli, M Carrie, Spencer, Melissa J, Sweeney, H Lee, Vetrone, Sylvia   +7 more
core   +2 more sources

Data on uncoupling protein-3 levels, hypoxia, low flow ischemia, and insulin stimulation in dystrophin-deficient mdx mouse hearts

Data in Brief, 2018
The data contain body weights, plasma free fatty acids concentrations and cardiac uncoupling protein-3 protein levels for wild-type and mdx mice. The data provide heart rates, left ventricular contractile functions, coronary flow, phosphocreatine ...
Wen Zhang, A. Elizabeth Sang, Michiel ten Hove, Stefan Neubauer, Kieran Clarke   +4 more
doaj   +1 more source

Notch signaling deficiency underlies age-dependent depletion of satellite cells in muscular dystrophy

Disease Models & Mechanisms, 2014
Duchenne muscular dystrophy (DMD) is a devastating disease characterized by muscle wasting, loss of mobility and death in early adulthood. Satellite cells are muscle-resident stem cells responsible for the repair and regeneration of damaged muscles.
Chunhui Jiang, Yefei Wen, Kazuki Kuroda, Kevin Hannon, Michael A. Rudnicki, Shihuan Kuang   +5 more
doaj   +1 more source

Are mice good models for human neuromuscular disease? Comparing muscle excursions in walking between mice and humans [PDF]

, 2017
The mouse is one of the most widely used animal models to study neuromuscular diseases and test new therapeutic strategies. However, findings from successful pre-clinical studies using mouse models frequently fail to translate to humans due to various ...
A De Luca, A Rajagopal, A Soman, AA Biewener, AA Biewener, AC Ludolph, AEH Emery, AL Camp, AV Birn-Jeffery, BH Wokke, BJ Petrof, C Pastoret, C Webster, CM Consolino, CM Eng, DM Escolar, E Azizi, E Mok, EK Moo, EM Arnold, EM Arnold, EM Arnold, FA Jenkins, FE Zajac, GM Buyse, GT Carter, H Radley-Crabb, H Stedman, HB van der Worp, HM Hsieh-Li, IG Kirkinezos, J Manning, JA Granchelli, JA Vilensky, JH Veldink, JJ Kaczor, JK Mah, JN Kornegay, JN Kornegay, JP Charles, JP Charles, JR Chamberlain, JW Carnwath, JW McGreevy, KA Clarke, KN An, L Xu, LM Ittner, M Benatar, M Bodor, M Millard, MD Grounds, MD Klein Horsman, ME Gurney, ME Llewellyn, MP Kadaba, N Alexander, N Serradj, NP Whitehead, P DeVita, P Moens, P Sicinski, R Burdi, R Willmann, R Willmann, RH Miller, RW Burgess, SL Delp, SL Delp, SR Ward, SS Blemker, SV Brooks, T Akay, TA McMahon, TA Partridge, TS Buchanan, V Brussee, VC Henderson, X Chen, X Chen, X Hu, ZJ Domire   +81 more
core   +3 more sources

T-Cell-Dependent Fibrosis in the mdx Dystrophic Mouse [PDF]

Laboratory Investigation, 2000
In Duchenne muscular dystrophy patients, the pathological hallmark of the disease, namely, the chronic accumulation of sclerotic scar tissue in the interstitial space of skeletal muscle is attributed to manifestation of secondary pathological processes.
Morrison, J, Lu, Q, Pastoret, C, Partridge, T, Bou-Gharios, G   +4 more
openaire   +2 more sources

Pharmacological inhibition of PKCθ counteracts muscle disease in a mouse model of duchenne muscular dystrophy [PDF]

, 2017
Inflammation plays a considerable role in the progression of Duchenne Muscular Dystrophy (DMD), a severe muscle disease caused by a mutation in the dystrophin gene. We previously showed that genetic ablation of Protein Kinase C θ (PKCθ) in mdx, the mouse
Benedetti, Anna, Bouche', Marina, Fiore, PIERA FILOMENA, LOZANOSKA OCHSER, Biliana, Madaro, Luca, Marrocco, Valeria, Musaro', Antonio, Pisu, Simona, Rizzuto, Emanuele   +8 more
core   +2 more sources

Microtubule-Mediated Misregulation of Junctophilin-2 Underlies T-Tubule Disruptions and Calcium Mishandling in mdx Mice

JACC: Basic to Translational Science, 2016
Cardiac myocytes from the mdx mouse, the mouse model of Duchenne muscular dystrophy, exhibit t-tubule disarray and increased calcium sparks, but a unifying molecular mechanism has not been elucidated.
Kurt W. Prins, MD, PhD, Michelle L. Asp, PhD, Huiliang Zhang, PhD, Wang Wang, MD, PhD, Joseph M. Metzger, PhD   +4 more
doaj   +1 more source

Lipidomic Analyses Reveal Specific Alterations of Phosphatidylcholine in Dystrophic Mdx Muscle

Frontiers in Physiology, 2022
In Duchenne muscular dystrophy (DMD), lack of dystrophin increases the permeability of myofiber plasma membranes to ions and larger macromolecules, disrupting calcium signaling and leading to progressive muscle wasting. Although the biological origin and
William J. Valentine, William J. Valentine, Sherif A. Mostafa, Sherif A. Mostafa, Suzumi M. Tokuoka, Fumie Hamano, Fumie Hamano, Natsuko F. Inagaki, Joel Z. Nordin, Joel Z. Nordin, Norio Motohashi, Yoshihiro Kita, Yoshitsugu Aoki, Takao Shimizu, Hideo Shindou, Hideo Shindou   +15 more
doaj   +1 more source

Differential requirement for utrophin in the induced pluripotent stem cell correction of muscle versus fat in muscular dystrophy mice. [PDF]

PLoS ONE, 2011
Duchenne muscular dystrophy (DMD) is an incurable degenerative muscle disorder. We injected WT mouse induced pluripotent stem cells (iPSCs) into mdx and mdx∶utrophin mutant blastocysts, which are predisposed to develop DMD with an increasing degree of ...
Amanda J Beck, Joseph M Vitale, Qingshi Zhao, Joel S Schneider, Corey Chang, Aneela Altaf, Jennifer Michaels, Mantu Bhaumik, Robert Grange, Diego Fraidenraich   +9 more
doaj   +1 more source