Viscoelastic response ultrasound for interrogating dystrophic muscle degeneration

2021 ◽  
Vol 150 (4) ◽  
pp. A288-A288
Author(s):  
Caterina M. Gallippi ◽  
Joseph B. Richardson ◽  
Christopher J. Moore ◽  
James F. Howard, Jr.
Keyword(s):  
Viscoelastic Response ◽  
Muscle Degeneration ◽  
Dystrophic Muscle

scholarly journals Oxidative Stress-Mediated Skeletal Muscle Degeneration: Molecules, Mechanisms, and Therapies

2016 ◽  
Vol 2016 ◽  
pp. 1-13 ◽  
Author(s):  
Min Hee Choi ◽  
Jin Rong Ow ◽  
Nai-Di Yang ◽  
Reshma Taneja
Keyword(s):  
Oxidative Stress ◽  
Molecular Level ◽  
Redox Homeostasis ◽  
Redox Status ◽  
Muscle Degeneration ◽  
Dystrophic Muscle ◽  
Telomere Shortening ◽  
Cellular Redox ◽  
Pathological Conditions

Oxidative stress is a loss of balance between the production of reactive oxygen species during cellular metabolism and the mechanisms that clear these species to maintain cellular redox homeostasis. Increased oxidative stress has been associated with muscular dystrophy, and many studies have proposed mechanisms that bridge these two pathological conditions at the molecular level. In this review, the evidence indicating a causal role of oxidative stress in the pathogenesis of various muscular dystrophies is revisited. In particular, the mediation of cellular redox status in dystrophic muscle by NF-κB pathway, autophagy, telomere shortening, and epigenetic regulation are discussed. Lastly, the current stance of targeting these pathways using antioxidant therapies in preclinical and clinical trials is examined.

scholarly journals NOX4 inhibition promotes the remodeling of dystrophic muscle

2022 ◽  
Author(s):  
David W Hammers
Keyword(s):  
Muscle Regeneration ◽  
Muscular Dystrophies ◽  
Advanced Stage ◽  
Effective Strategy ◽  
Muscle Degeneration ◽  
Regenerative Processes ◽  
Dystrophic Muscle ◽  
Stage Disease ◽  
Potential Strategy ◽  
Dystrophic Mice

The muscular dystrophies (MDs) are genetic muscle diseases that result in progressive muscle degeneration followed by the fibrotic replacement of affected muscles as regenerative processes fail. Therapeutics that specifically address the fibrosis and failed regeneration associated with MDs represent a major unmet clinical need for MD patients, particularly those with advanced stage disease progression. The current study investigates targeting NAD(P)H oxidase (NOX) 4 as a potential strategy to reduce fibrosis and promote regeneration in disease-burdened muscle that models Duchenne muscular dystrophy (DMD). NOX4 is elevated in the muscles of dystrophic mice and DMD patients, localizing primarily to interstitial cells located between muscle fibers. Genetic and pharmacological targeting of NOX4 significantly reduces fibrosis in dystrophic respiratory and limb muscles. Mechanistically, NOX4 targeting decreases the number of fibrosis-depositing cells (myofibroblasts) and restores the number of muscle-specific stem cells (satellite cells) to their physiological niche, thereby, rejuvenating muscle regeneration. Furthermore, acute inhibition of NOX4 is sufficient to induce apoptotic clearing of myofibroblasts within dystrophic muscle. These data indicate that targeting NOX4 is an effective strategy to promote the beneficial remodeling of disease-burdened muscle representative of DMD and, potentially, other MDs and muscle pathologies.

scholarly journals α1-Syntrophin Gene Disruption Results in the Absence of Neuronal-type Nitric-oxide Synthase at the Sarcolemma but Does Not Induce Muscle Degeneration

1999 ◽  
Vol 274 (4) ◽  
pp. 2193-2200 ◽  
Author(s):  
Shuhei Kameya ◽  
Yuko Miyagoe ◽  
Ikuya Nonaka ◽  
Takaaki Ikemoto ◽  
Makoto Endo ◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Nitric Oxide Synthase ◽  
Gene Disruption ◽  
Muscle Degeneration ◽  
Oxide Synthase ◽  
Neuronal Type

scholarly journals Roles for RNA export factor, Nxt1, in ensuring muscle integrity and normal RNA expression in Drosophila

2020 ◽  
Vol 11 (1) ◽  
Author(s):  
Kevin van der Graaf ◽  
Katia Jindrich ◽  
Robert Mitchell ◽  
Helen White-Cooper
Keyword(s):  
Mrna Export ◽  
Rna Stability ◽  
Muscle Degeneration ◽  
Loss Of Function ◽  
Cellular Functions ◽  
Partial Loss ◽  
Export Pathway ◽  
Pathway Gene ◽  
Rna Export

Abstract The mRNA export pathway is responsible for the transport of mRNAs from the nucleus to the cytoplasm, and thus is essential for protein production and normal cellular functions. A partial loss of function allele of the mRNA export factor Nxt1 in Drosophila shows reduced viability and sterility. A previous study has shown that the male fertility defect is due to a defect in transcription and RNA stability, indicating the potential for this pathway to be implicated in processes beyond the known mRNA transport function. Here we investigate the reduced viability of Nxt1 partial loss of function mutants, and describe a defect in growth and maintenance of the larval muscles, leading to muscle degeneration. RNA-seq revealed reduced expression of a set of mRNAs, particularly from genes with long introns in Nxt1 mutant carcass. We detected differential expression of circRNA, and significantly fewer distinct circRNAs expressed in the mutants. Despite the widespread defects in gene expression, muscle degeneration was rescued by increased expression of the costamere component tn (abba) in muscles. This is the first report of a role for the RNA export pathway gene Nxt1 in the maintenance of muscle integrity. Our data also links the mRNA export pathway to a specific role in the expression of mRNA and circRNA from common precursor genes, in vivo.

scholarly journals Is there a correlation between upper lumbar disc herniation and multifidus muscle degeneration? A retrospective study of MRI morphology

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Chong Liu ◽  
Jiang Xue ◽  
Jingjing Liu ◽  
Gang Ma ◽  
Abu Moro ◽  
...  
Keyword(s):  
Lumbar Disc Herniation ◽  
Lumbar Disc ◽  
Fatty Infiltration ◽  
Cross Sectional Area ◽  
Sectional Area ◽  
Muscle Degeneration ◽  
Multifidus Muscle ◽  
Cross Sectional ◽  
Upper Lumbar Disc Herniation ◽  
Muscle Cross Sectional Area

Abstract Background The purpose of the study is to investigate the correlation between upper lumbar disc herniation (ULDH) and multifidus muscle degeneration via the comparison of width, the cross-sectional area and degree of fatty infiltration of the lumbar multifidus muscle. Methods Using the axial T2-weighted images of magnetic resonance imaging as an assessment tool, we retrospectively investigated 132 patients with ULDH and 132 healthy individuals. The total muscle cross-sectional area (TMCSA) and the pure muscle cross-sectional area (PMCSA) of the multifidus muscle at the L1/2, L2/3, and L3/4 intervertebral disc levels were measured respectively, and in the meantime, the average multifidus muscle width (AMMW) and degree of fatty infiltration of bilateral multifidus muscle were evaluated. The resulting data were analyzed to determine the presence/absence of statistical significance between the study and control groups. Multivariate logistical regression analyses were used to evaluate the correlation between ULDH and multifidus degeneration. Results The results of the analysis of the two groups showed that there were statistically significant differences (p < 0.05) between TMCSA, PMCSA, AMMW and degree of fatty infiltration. The multivariate logistic regression analysis indicated that the TMCSA, PMCSA, AMMW and the degree of fatty infiltration of multifidus muscle were correlated with ULDH, and the differences were statistically significant (P < 0.05). Conclusions A correlation could exist between multifidus muscles degeneration and ULDH, that may be a process of mutual influence and interaction. Lumbar muscle strengthening training could prevent and improve muscle atrophy and degeneration.

scholarly journals Muscle Degeneration: Preparation of Stretchable Nanofibrous Sheets with Sacrificial Coaxial Electrospinning for Treatment of Traumatic Muscle Injury (Adv. Healthcare Mater. 8/2021)

2021 ◽  
Vol 10 (8) ◽  
pp. 2170041
Author(s):  
Oanh‐Vu Pham‐Nguyen ◽  
Young Ju Son ◽  
Tae‐wan Kwon ◽  
Junhyung Kim ◽  
Yun Chan Jung ◽  
...  
Keyword(s):  
Muscle Injury ◽  
Coaxial Electrospinning ◽  
Muscle Degeneration
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