Results 71 to 80 of about 28,350 (246)
Increased neointimal thickening in dystrophin-deficient mdx mice.
PLoS ONE, 2012 BackgroundThe dystrophin gene, which is mutated in Duchenne muscular dystrophy (DMD), encodes a large cytoskeletal protein present in muscle fibers. While dystrophin in skeletal muscle has been extensively studied, the function of dystrophin in vascular ...
Uwe Rauch +5 more
doaj +1 more source
Proteomic Profiling of Myofiber Repair Annexins and Their Role in Duchenne Muscular Dystrophy
PROTEOMICS, EarlyView.ABSTRACT Myofiber regeneration and membrane repair play crucial roles in maintaining the continuous physiological functioning of the neuromuscular system. A swift and efficient repair mechanism enables the rapid restoration of sarcolemmal integrity following cellular impairment in damaged skeletal muscles.
Paul Dowling +6 more
wiley +1 more source
U7 snRNAs induce correction of mutated dystrophin pre-mRNA by exon skipping. [PDF]
, 2003 Most cases of Duchenne muscular dystrophy are caused by dystrophin gene mutations that disrupt the mRNA reading frame. Artificial exclusion (skipping) of a single exon would often restore the reading frame, giving rise to a shorter, but still functional ...
Brun, C +7 more
core
, 2013 Representative images of dystrophin immunohistochemistry (brown staining) in skeletal muscle counterstained with haematoxylin and eosin from (A) a control horse and (B) a horse heterozygous for the GYS1 mutation. Note the normal dystrophin expression and
Nicole Henke (313693) +7 more
core +1 more source
Developmental Medicine &Child Neurology, EarlyView.The Brain Involvement iN Dystrophinopathies (BIND) screener is an 18‐item questionnaire with strong reliability and validity for identifying potential brain‐related comorbidities in Duchenne muscular dystrophy. It allows rapid, cross‐age and cross‐country screening for both clinical and research purposes, demonstrating good sensitivity and specificity.
Ruben Miranda +46 more
wiley +1 more source
The FEBS Journal, EarlyView.Alpha‐crystallin B chain (CRYAB) has been reported to stabilize mothers against decapentaplegic homolog 4 (SMAD4) in transforming growth factor‐β (TGF‐β) signaling, enabling target gene transcription. We show nuclear CRYAB–SMAD4 interaction for the first time in human skeletal muscle fibers and its regulation by exercise.
Kirill Schaaf +7 more
wiley +1 more source
Dystrophin signal specificity using MANDYS106.
, 2014 Immunofluorescence intensities measured in pre-treatment biopsies of different patients with DMD at microscope settings suitable for imaging DMD samples (7% laser intensity). A. (Left): Dystrophin staining (AlexaFluor488).
Judith C. van Deutekom (632518) +8 more
core +1 more source
One day of environment‐induced heat stress causes injury to the murine kidney
Experimental Physiology, EarlyView.Abstract Environment‐induced heat stress (EIHS) results from sustained body temperature elevation owing to prolonged exposure to heat and humidity. We hypothesized that EIHS would cause kidney injury and cellular dysfunction. To test this hypothesis, female C57 mice were exposed to EIHS (n = 14; 37.6°C, 42.0% relative humidity) or thermoneutral (TN ...
Melissa Roths +3 more
wiley +1 more source
, 2010 Duchenne muscular dystrophy (DMD) is characterised by the absence of dystrophin in muscle biopsies, although residual dystrophin can be present, either as dystrophin-positive (revertant) fibres or traces.
Main, M +12 more
core
N-terminal domain of dystrophin
, 1994 Contro-versial experiments have been published on calmodulin binding of dystrophin. In this study, we used recombinant proteins and the techniques of affinity chromatography and ELISA to show that the N-terminal part of dystrophin binds calmodulin ...
Fabbrizio, Eric +5 more
core +1 more source