Towards developing standard operating procedures for pre-clinical testing in the mdx mouse model of Duchenne muscular dystrophy

MD Grounds, HG Radley, GS Lynch, K Nagaraju… - Neurobiology of …, 2008 - Elsevier
This review discusses various issues to consider when developing standard operating
procedures for pre-clinical studies in the mdx mouse model of Duchenne muscular …

[HTML][HTML] Synaptic alterations as a neurodevelopmental trait of Duchenne muscular dystrophy

ME De Stefano, V Ferretti, C Mozzetta - Neurobiology of Disease, 2022 - Elsevier
Dystrophinopaties, eg, Duchenne muscular dystrophy (DMD), Becker muscular dystrophy
and X-linked dilated cardiomyopathy are inherited neuromuscular diseases, characterized …

Uncovering molecular biomarkers that correlate cognitive decline with the changes of hippocampus' gene expression profiles in Alzheimer's disease

M Gomez Ravetti, OA Rosso, R Berretta, P Moscato - PloS one, 2010 - journals.plos.org
Background Alzheimer's disease (AD) is characterized by a neurodegenerative progression
that alters cognition. On a phenotypical level, cognition is evaluated by means of the …

Cycles of myofiber degeneration and regeneration lead to remodeling of the neuromuscular junction in two mammalian models of Duchenne muscular dystrophy

SG Haddix, YI Lee, JN Kornegay, WJ Thompson - PLoS One, 2018 - journals.plos.org
Mice lacking the sarcolemmal protein dystrophin, designated mdx, have been widely used
as a model of Duchenne muscular dystrophy. Dystrophic mdx mice as they mature develop …

Recent insights into neuromuscular junction biology in Duchenne muscular dystrophy: Impacts, challenges, and opportunities

SY Ng, V Ljubicic - EBioMedicine, 2020 - thelancet.com
Duchenne muscular dystrophy (DMD) is the most common and relentless form of muscular
dystrophy. The pleiotropic effects of dystrophin deficiency include remarkable impacts on …

Truncated dystrophins can influence neuromuscular synapse structure

GB Banks, JS Chamberlain, SC Froehner - Molecular and Cellular …, 2009 - Elsevier
Duchenne muscular dystrophy (DMD) is characterized by muscle degeneration and
structural defects in the neuromuscular synapse that are caused by mutations in dystrophin …

RNAi-mediated knockdown of dystrophin expression in adult mice does not lead to overt muscular dystrophy pathology

MM Ghahramani Seno, IR Graham… - Human molecular …, 2008 - academic.oup.com
Duchenne muscular dystrophy (DMD) is a fatal muscle wasting disorder caused by
mutations in the dystrophin gene. DMD has a complex and as yet incompletely defined …

Heterologous fibrin biopolymer associated to a single suture stitch enables the return of neuromuscular junction to its mature pattern after peripheral nerve injury

CG Pinto, APS Leite, AA Sartori, FC Tibúrcio… - Injury, 2021 - Elsevier
Denervation leads to severe atrophy of neuromuscular junction (NMJ) structure including
decrease of the expression of fundamental proteins. Up to now, conventional suture has …

Cilostazol attenuates oxidative stress and apoptosis in the quadriceps muscle of the dystrophic mouse experimental model

TA Hermes, RD Mâncio, DS Mizobutti… - International journal …, 2023 - Wiley Online Library
Duchenne muscular dystrophy (DMD) is the most severe and frequent form of muscular
dystrophy. The mdx mouse is one of the most widely used experimental models to …

Fiber Type Composition of the Sternomastoid and Diaphragm Muscles of Dystrophin‐Deficient mdx Mice

AN Guido, GER Campos, HS Neto… - … Record: Advances in …, 2010 - Wiley Online Library
The muscle fiber phenotype is mainly determined by motoneuron innervation and changes
in neuromuscular interaction alter the muscle fiber type. In dystrophin‐deficient mdx mice …