scholarly journals Gene expression comparison of biopsies from Duchenne muscular dystrophy (DMD) and normal skeletal muscle

2002 ◽  
Vol 99 (23) ◽  
pp. 15000-15005 ◽  
Author(s):  
J. N. Haslett ◽  
D. Sanoudou ◽  
A. T. Kho ◽  
R. R. Bennett ◽  
S. A. Greenberg ◽  
...  
Keyword(s):  
Gene Expression ◽  
Skeletal Muscle ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy

Gene expression profiling of Duchenne muscular dystrophy skeletal muscle

Neurogenetics ◽  
2003 ◽  
Vol 4 (4) ◽  
pp. 163-171 ◽  
Author(s):  
Judith N. Haslett ◽  
Despina Sanoudou ◽  
Alvin T. Kho ◽  
Mei Han ◽  
Richard R. Bennett ◽  
...  
Keyword(s):  
Gene Expression ◽  
Skeletal Muscle ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Gene Expression Profiling ◽  
Expression Profiling

scholarly journals 34. Phi C31 Integrase System Enhances Dystrophin Gene Expression in Skeletal Muscle of Mouse Models for Duchenne Muscular Dystrophy

2006 ◽  
Vol 13 ◽  
pp. S14-S15
Author(s):  
Carmen Bertoni ◽  
Sohail Jarrahian ◽  
Thurman M. Wheeler ◽  
Yining Li ◽  
Eric C. Olivares ◽  
...  
Keyword(s):  
Gene Expression ◽  
Skeletal Muscle ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Mouse Models ◽  
Dystrophin Gene

scholarly journals Gene expression effects of glucocorticoid and mineralocorticoid receptor agonists and antagonists on normal human skeletal muscle

2017 ◽  
Vol 49 (6) ◽  
pp. 277-286 ◽  
Author(s):  
Jessica A. Chadwick ◽  
J. Spencer Hauck ◽  
Celso E. Gomez-Sanchez ◽  
Elise P. Gomez-Sanchez ◽  
Jill A. Rafael-Fortney
Keyword(s):  
Gene Expression ◽  
Skeletal Muscle ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Skeletal Muscles ◽  
Human Skeletal Muscle ◽  
Expression Profiles ◽  
Combined Treatment ◽  
Gene Expression Profiles ◽  
Steroid Hormone Receptors

Mineralocorticoid and glucocorticoid receptors are closely related steroid hormone receptors that regulate gene expression through many of the same hormone response elements. However, their transcriptional activities and effects in skeletal muscles are largely unknown. We recently identified mineralocorticoid receptors (MR) in skeletal muscles after finding that combined treatment with the angiotensin-converting enzyme inhibitor lisinopril and MR antagonist spironolactone was therapeutic in Duchenne muscular dystrophy mouse models. The glucocorticoid receptor (GR) agonist prednisolone is the current standard-of-care treatment for Duchenne muscular dystrophy because it prolongs ambulation, likely due to its anti-inflammatory effects. However, data on whether glucocorticoids have a beneficial or detrimental direct effect on skeletal muscle are controversial. Here, we begin to define the gene expression profiles in normal differentiated human skeletal muscle myotubes treated with MR and GR agonists and antagonists. The MR agonist aldosterone and GR agonist prednisolone had highly overlapping gene expression profiles, supporting the notion that prednisolone acts as both a GR and MR agonist that may have detrimental effects on skeletal muscles. Co-incubations with aldosterone plus either nonspecific or selective MR antagonists, spironolactone or eplerenone, resulted in similar numbers of gene expression changes, suggesting that both drugs can block MR activation to a similar extent. Eplerenone treatment alone decreased a number of important muscle-specific genes. This information may be used to develop biomarkers to monitor clinical efficacy of MR antagonists or GR agonists in muscular dystrophy, develop a temporally coordinated treatment with both drugs, or identify novel therapeutics with more specific downstream targets.

scholarly journals Autophagy in the heart is enhanced and independent of disease progression in mus musculus dystrophinopathy models

2019 ◽  
Vol 8 ◽  
pp. 204800401987958
Author(s):  
HR Spaulding ◽  
C Ballmann ◽  
JC Quindry ◽  
MB Hudson ◽  
JT Selsby
Keyword(s):  
Skeletal Muscle ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Disease Progression ◽  
Gene Mutations ◽  
Dystrophin Gene ◽  
Mdx Mice ◽  
Dystrophin Deficiency ◽  
Muscle Wasting Disease ◽  
Dystrophin Protein

Background Duchenne muscular dystrophy is a muscle wasting disease caused by dystrophin gene mutations resulting in dysfunctional dystrophin protein. Autophagy, a proteolytic process, is impaired in dystrophic skeletal muscle though little is known about the effect of dystrophin deficiency on autophagy in cardiac muscle. We hypothesized that with disease progression autophagy would become increasingly dysfunctional based upon indirect autophagic markers. Methods Markers of autophagy were measured by western blot in 7-week-old and 17-month-old control (C57) and dystrophic (mdx) hearts. Results Counter to our hypothesis, markers of autophagy were similar between groups. Given these surprising results, two independent experiments were conducted using 14-month-old mdx mice or 10-month-old mdx/Utrn± mice, a more severe model of Duchenne muscular dystrophy. Data from these animals suggest increased autophagosome degradation. Conclusion Together these data suggest that autophagy is not impaired in the dystrophic myocardium as it is in dystrophic skeletal muscle and that disease progression and related injury is independent of autophagic dysfunction.

A carrier of Duchenne muscular dystrophy with dilated cardiomyopathy but no skeletal muscle symptom

1995 ◽  
Vol 17 (3) ◽  
pp. 202-205 ◽  
Author(s):  
Hirotoshi Kinoshita ◽  
Yu-ichi Goto ◽  
Mitsuru Ishikawa ◽  
Tetsuya Uemura ◽  
Kouichi Matsumoto ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Dilated Cardiomyopathy ◽  
Muscle Symptom

The Duchenne muscular dystrophy gene product is localized in sarcolemma of human skeletal muscle

Nature ◽  
1988 ◽  
Vol 333 (6172) ◽  
pp. 466-469 ◽  
Author(s):  
Elizabeth E. Zubrzycka-Gaarn ◽  
Dennis E. Bulman ◽  
George Karpati ◽  
Arthur H. M. Burghes ◽  
Bonnie Belfall ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Gene Product ◽  
Human Skeletal Muscle ◽  
Duchenne Muscular Dystrophy Gene

scholarly journals Abnormalities of Creatinase in Skeletal Muscle of Patients with Duchenne Muscular Dystrophy

1980 ◽  
Vol 56 (2) ◽  
pp. 99-101 ◽  
Author(s):  
Kazuo MIYOSHI ◽  
Akira TAIRA ◽  
Kenzo YOSHIDA ◽  
Katsuya TAMURA ◽  
Shigetoshi UGA
Keyword(s):  
Skeletal Muscle ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy
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