scholarly journals β-Glucans as Dietary Supplement to Improve Locomotion and Mitochondrial Respiration in a Model of Duchenne Muscular Dystrophy

Nutrients ◽  
2021 ◽  
Vol 13 (5) ◽  
pp. 1619
Author(s):  
Letizia Brogi ◽  
Maria Marchese ◽  
Alessandro Cellerino ◽  
Rosario Licitra ◽  
Valentina Naef ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Immune Responses ◽  
Mitochondrial Function ◽  
Mitochondrial Respiration ◽  
Life Quality ◽  
Adaptive Immune ◽  
Progressive Muscle Weakness ◽  
Diet Supplement ◽  
And Oxidative Stress

Duchenne muscular dystrophy (DMD) is a severe X-linked neuromuscular childhood disorder that causes progressive muscle weakness and degeneration. A lack of dystrophin in DMD leads to inflammatory response, autophagic dysregulation, and oxidative stress in skeletal muscle fibers that play a key role in the progression of the pathology. β-glucans can modulate immune function by modifying the phagocytic activity of immunocompetent cells, notably macrophages. Mitochondrial function is also involved in an important mechanism of the innate and adaptive immune responses, owing to high need for energy of immune cells. In the present study, the effects of 1,3-1,6 β-glucans on five-day-old non-dystrophic and dystrophic (sapje) zebrafish larvae were investigated. The effects of the sonication of β-glucans and the dechorionation of embryos were also evaluated. The results showed that the incidence of dystrophic phenotypes was reduced when dystrophic embryos were exposed to 2 and 4 mg L−1 of 1,3-1,6 β-glucans. Moreover, when the dystrophic larvae underwent 8 mg L−1 treatment, an improvement of the locomotor performances and mitochondrial respiration were observed. In conclusion, the observed results demonstrated that 1,3-1,6 β-glucans improve locomotor performances and mitochondrial function in dystrophic zebrafish. Therefore, for ameliorating their life quality, 1,3-1,6 β-glucans look like a promising diet supplement for DMD patients, even though further investigations are required.

scholarly journals Duchenne Muscular Dystrophy - Family in a Crisis

1970 ◽  
pp. 36-39
Author(s):  
M Robed Amin ◽  
Chowdhury Chironjib Borua ◽  
Kaji Shafiqul Alam ◽  
Fazle Rabbi Chowdhury ◽  
Rabiul Jahan Sarkar ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Case Series ◽  
Dystrophin Gene ◽  
Muscle Disease ◽  
Motor Neuron Diseases ◽  
Muscle Diseases ◽  
Progressive Muscle Weakness ◽  
Recessive Disorders ◽  
Dystrophin Protein

Progressive muscular weakness with deformity leading to crippled states develop due to musculoskeletal and neurological disorders. Sometimes it is difficult to differentiate between primary muscle disease and neurological disease. But there is some classical presentation of muscle diseases which have its own entity and thus can be clinically differentiated from neurological disorder especially spinal cord and motor neuron diseases. Muscular dystrophy is one of those disorder with distinct clinical features. Muscular dystrophy refers to a group of genetic, hereditary muscle diseases that cause progressive muscle weakness. Most types of MD are multi-system disorders with manifestations in body systems including skeletal system, the heart, gastrointestinal and nervous systems, endocrine glands, skin, eyes and other organs. Duchenne muscular dystrophy (DMD), is inherited in an X-linked recessive pattern, meaning that the mutated gene that causes the disorder is located on the X chromosome, one of the two sex chromosomes, and is thus considered sex-linked. Males are therefore affected by X-linked recessive disorders much more often than females. A characteristic of X-linked inheritance is that fathers cannot pass X-linked traits to their sons. Duchenne muscular dystrophy and Backers muscular dystrophy are caused by mutations of the gene for the dystrophin protein and lead to an overabundance of the enzyme creatine kinase. The dystrophin gene is the largest gene in humans. In this case series a family with three brothers suffering from Duchenne muscular dystrophy is described and review with literature was done.   doi:10.3329/jom.v10i3.2015 J Medicine 2009; 10 (Supplement 1): 36-39

scholarly journals Selective PPARδ Modulators Improve Mitochondrial Function: Potential Treatment for Duchenne Muscular Dystrophy (DMD)

2018 ◽  
Vol 9 (9) ◽  
pp. 935-940 ◽  
Author(s):  
Bharat Lagu ◽  
Arthur F. Kluge ◽  
Effie Tozzo ◽  
Ross Fredenburg ◽  
Eric L. Bell ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Mitochondrial Function ◽  
Potential Treatment ◽  
Function Potential

scholarly journals Diagnosis of Duchenne Muscular Dystrophy using Raman Hyperspectroscopy

2020 ◽  
Author(s):  
Nicole M. Ralbovsky ◽  
Paromita Dey ◽  
Andrew Galfano ◽  
Bijan K. Dey ◽  
Igor K. Lednev
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Blood Serum ◽  
External Validation ◽  
Diagnostic Algorithm ◽  
Severe Form ◽  
Specific Method ◽  
Muscle Degeneration ◽  
Treatment Regimens ◽  
Progressive Muscle Weakness

AbstractDuchenne muscular dystrophy (DMD) is the most common and severe form of muscular dystrophy and affects boys in infancy or early childhood. DMD is known to trigger progressive muscle weakness due to skeletal muscle degeneration and ultimately causes death. There are limited treatment regimens available that can either slow or stop the progression of DMD. An accurate and specific method for diagnosing DMD in its earliest stages is needed to prevent progressive muscle degeneration and death. Current methods for diagnosing DMD are often laborious, expensive, invasive, and typically diagnose the disease later on it is progression. In an effort to improve the accuracy and ease of diagnosis, this study focused on developing a novel method for diagnosing DMD which combines Raman hyperspectroscopic analysis of blood serum with advanced statistical analysis. Partial Least Squares Discriminant Analysis (PLS-DA), was applied to the spectral dataset acquired from control and mdx blood serum of 3- and 12-month old mice to build a diagnostic algorithm. Internal cross-validation showed 95.2% sensitivity and 94.6% specificity for identifying diseased spectra. These results were verified using external validation, which achieved 100% successful classification efficiency at the level of individual donor. This proof-of-concept study presents Raman hyperspectroscopic analysis of blood serum as a fast, non-expensive, minimally invasive and early detection method for the diagnosis of Duchenne muscular dystrophy.

scholarly journals A COMPARATIVE AYURVEDIC REVIEW OF ETIOPATHOGENESIS OF DUCHENNE MUSCULAR DYSTROPHY (INHERITED DISORDER)

2021 ◽  
Vol 12 (1) ◽  
pp. 124-125
Author(s):  
Ravi Shankar Khatri ◽  
Mridul Ranajan ◽  
Shalini .
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Muscle Weakness ◽  
Inherited Diseases ◽  
Progressive Muscle Weakness

Duchenne muscular dystrophy (DMD) is an inherited disorder with severe progressive muscle weakness. In Ayurveda, Adibala Pravritta Vyadhi are also known as inherited diseases that caused by Matruja beeja dushti (Shonita) and Pitruja beeja dushti (Shukra). Duchenne muscular dystrophy (DMD) has been classified under Adibala Pravritta Vyadhi as per Ayurveda. The main objective of this article is to describe the various aspect of etiopathogenesis of Duchenne muscular dystrophy (DMD) as per Ayurvedic literature. This article will be helpful to making the Nidana (Diagnosis) as per Ayurveda and also help in the Chikitsa (Treatment) of Duchenne muscular dystrophy.

L-type Ca2+ channel contributes to alterations in mitochondrial calcium handling in the mdx ventricular myocyte

2013 ◽  
Vol 304 (6) ◽  
pp. H767-H775 ◽  
Author(s):  
Helena M. Viola ◽  
Stefan M. K. Davies ◽  
Aleksandra Filipovska ◽  
Livia C. Hool
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Mitochondrial Function ◽  
Ventricular Myocytes ◽  
Chain Complex ◽  
Cytoskeletal Proteins ◽  
Calcium Handling ◽  
Complex Activity ◽  
Whole Cell ◽  
Channel Current

The L-type Ca2+ channel is the main route for calcium entry into cardiac myocytes, and it is essential for contraction. Alterations in whole cell L-type Ca2+ channel current and Ca2+ homeostasis have been implicated in the development of cardiomyopathies. Cytoskeletal proteins can influence whole cell L-type Ca2+ current and mitochondrial function. Duchenne muscular dystrophy is a fatal X-linked disease that leads to progressive muscle weakness due to the absence of cytoskeletal protein dystrophin. This includes dilated cardiomyopathy, but the mechanisms are not well understood. We sought to identify the effect of alterations in whole cell L-type Ca2+ channel current on mitochondrial function in the murine model of Duchenne muscular dystrophy ( mdx). Activation of the L-type Ca2+ channel with the dihydropyridine agonist BayK(−) caused a significantly larger increase in cytosolic Ca2+ in mdx vs. wild-type ( wt) ventricular myocytes. Consistent with elevated cytosolic Ca2+, resting mitochondrial Ca2+, NADH, and mitochondrial superoxide were significantly greater in mdx vs. wt myocytes. Activation of the channel with BayK(−) caused a further increase in mitochondrial Ca2+, NADH, and superoxide in mdx myocytes. The ratios of the increases were similar to the ratios recorded in wt myocytes. In mitochondria isolated from 8-wk-old mdx hearts, respiration and mitochondrial electron transport chain complex activity were similar to mitochondria isolated from wt hearts. We conclude that mitochondria function at a higher level of resting calcium in the intact mdx myocyte and activation of the L-type Ca2+ channel contributes to alterations in calcium handling by the mitochondria. This perturbation may contribute to the development of cardiomyopathy.

scholarly journals Optimizing Bone Health in Duchenne Muscular Dystrophy

2015 ◽  
Vol 2015 ◽  
pp. 1-9 ◽  
Author(s):  
Jason L. Buckner ◽  
Sasigarn A. Bowden ◽  
John D. Mahan
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Bone Mass ◽  
Bone Health ◽  
Fragility Fractures ◽  
Premature Death ◽  
Screening Methods ◽  
Mineral Density ◽  
Progressive Muscle Weakness ◽  
Spinal Imaging

Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder characterized by progressive muscle weakness, with eventual loss of ambulation and premature death. The approved therapy with corticosteroids improves muscle strength, prolongs ambulation, and maintains pulmonary function. However, the osteoporotic impact of chronic corticosteroid use further impairs the underlying reduced bone mass seen in DMD, leading to increased fragility fractures of long bones and vertebrae. These serious sequelae adversely affect quality of life and can impact survival. The current clinical issues relating to bone health and bone health screening methods in DMD are presented in this review. Diagnostic studies, including biochemical markers of bone turnover and bone mineral density by dual energy X-ray absorptiometry (DXA), as well as spinal imaging using densitometric lateral spinal imaging, and treatment to optimize bone health in patients with DMD are discussed. Treatment with bisphosphonates offers a method to increase bone mass in these children; oral and intravenous bisphosphonates have been used successfully although treatment is typically reserved for children with fractures and/or bone pain with low bone mass by DXA.

scholarly journals Swimming Prevents Memory Impairment by Increasing the Antioxidant Defense in an Animal Model of Duchenne Muscular Dystrophy

2019 ◽  
Author(s):  
Priscila Mantovani Nocetti Ribeiro ◽  
Adriano Alberti ◽  
Viviane Freiberger ◽  
Letícia Ventura ◽  
Leoberto Ricardo Grigollo ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Lipid Peroxidation ◽  
Animal Model ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Antioxidant Defense ◽  
Antioxidant Defenses ◽  
Mdx Mice ◽  
Encephalic Structures ◽  
And Oxidative Stress

Duchenne muscular dystrophy (DMD) is a genetic disease which is associated to a progressive skeletical muscle degeneration. Swimming is usually indicated for avoiding impact and facilitating adherence because of a better adaptation to a warm water invironment and also for its benefits on cognition, and modulating memory and learning processes and for increasing antioxidant defenses in oxidative stress. The objective of this study was to evaluate the effects of a swimming protocol on memory and oxidative stress in an animal model of Duchenne muscular dystrophy. Methods: male mdx and wild type mice within 28 days were used in this study. The animals were trained in an stepped swimming protocol for four consecutive weeks. Twenty four hours after the last exercise day, aversive memory and habituation memory tests were performed and removed the encephalic structures of striatus, pre frontal cortex, hippocampus, and cortex and gastrocnemius and diafragma muscles to evaluate protein carbonilation and lipid peroxidation and free thiols. Results: it was verified that swimming was able to reduce significantly the levels of lipid peroxidation and protein carbonilation in gastrocnemius and hippocampus and striatus in exercised animals. Swimming has also prevented lipid peroxidation in diafragma. Besides, this swimming protocol was able to increase free thiols in gastrocnemius, diafragma and in analysed SNC structures. These results showed that swimming prevented aversive and habituation memory in mdx mice.

Alisporivir rescues defective mitochondrial respiration in Duchenne muscular dystrophy

2017 ◽  
Vol 125 ◽  
pp. 122-131 ◽  
Author(s):  
Marco Schiavone ◽  
Alessandra Zulian ◽  
Sara Menazza ◽  
Valeria Petronilli ◽  
Francesco Argenton ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Mitochondrial Respiration
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