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Electrical stimulation induces calcium-dependent up-regulation of neuregulin-1β in dystrophic skeletal muscle cell lines
(2012)
Duchenne muscular dystrophy (DMD) is a
neuromuscular disease originated by reduced or no
expression of dystrophin, a cytoskeletal protein that
provides structural integrity to muscle fibres. A
promising pharmacological ...
Abnormal distribution of inositol 1,4,5-trisphosphate receptors in human muscle can be related to altered calcium signals and gene expression in Duchenne dystrophy-derived cells
(2010)
Inositol 1,4,5-trisphosphate (IP3) receptors (IP3Rs) drive calcium signals involved in skeletal muscle excitation-transcription coupling and plasticity; IP3R subtype distribution and downstream events evoked by their ...
Distrofia muscular de Becker con duplicación en el exón 5del gen DMD
(Sociedad de Cirugía de Bogotá, Hospital de San José y Fundación Universitaria de Ciencias de la Salud, 2019-07-01)
Deleciones en el gen de la distrofina en 62 familias colombianas: correlación genotipofenotipo para la distrofia muscular de Duchenne y Becker.
(2013-11-30)
Introducción: La correlación genotipo-fenotipo se estableció mediante el análisis de
deleciones del gen de la distrofina en pacientes con distrofia muscular de Duchenne y
Becker (DMD/DMB).
Objetivos: Establecer la ...
Ataluren in patients with nonsense mutation Duchenne muscular dystrophy (ACT DMD): a multicentre, randomised, double blind, placebo controlled, phase 3 trial
(Elsevier, 2017)
Background Duchenne muscular dystrophy (DMD) is a severe, progressive, and rare neuromuscular, X-linked recessive disease. Dystrophin deficiency is the underlying cause of disease; therefore, mutation-specific therapies ...
A human skeletal-muscle cell-line obtained from an adult donor
(Elsevier, 1992)
A cell line (RCMH) in permanent culture was established from surgically removed adult normal human skeletal muscle by exposure to conditioned media obtained from thyroid cells. Cells proliferated indefinitely but displayed ...
Altered ROS production, NF-κB activation and interleukin-6 gene expression induced by electrical stimulation in dystrophic mdx skeletal muscle cells
(Elsevier, 2015)
Duchenne muscular dystrophy is a fatal X-linked genetic disease, caused by mutations in the dystrophin gene, which
cause functional loss of this protein. This pathology is associated with an increased production of reactive ...
Fast skeletal myofibers of mdx mouse, model of Duchenne muscular dystrophy, express connexin hemichannels that lead to apoptosis
(Springer, 2016)
Skeletal muscles of patients with Duchenne muscular dystrophy (DMD) show numerous alterations including inflammation, apoptosis, and necrosis of myofibers. However, the molecular mechanism that explains these changes remains ...