Congenital morphological patterns of myocardial bridges.

Background: Myocardial bridging (MB) is a coronary anomaly in which a segment of the coronary artery is overlapped by a layer of myocardial tissue. Nowadays, there is no scientific agreement on if the MB are congenital or acquired or on the factors that determine their presence and/or absence.

Objective: This study is performed to analyze the anatomical characteristics of adult and children's hearts regarding the shape of the left coronary artery branching, presence of pre-bridge arterial branch, coronary dominance and its correlations to MB formation.

Effect of Photobiomodulation on Denervation-Induced Skeletal Muscle Atrophy and Autophagy: A Study in Mice.

Objective: The purpose of this study was to investigate whether photobiomodulation (PBM) can protect against and attenuate muscle atrophy owing to complete peripheral nerve lesion in mice by acting on autophagy.

Methods: C57BL/10 mice underwent right sciatic nerve transection to induce tibialis anterior muscle atrophy. After 6 hours of denervation, the mice received PBM (wavelength, 830 nm) daily, transcutaneously over the tibialis anterior muscle region for 5 or 14 days.

Intrafamilial phenotypic heterogeneity related to a new DMD splice site variant.

Dystrophinopathies are a group of X-linked neuromuscular disorders that result from pathogenic variants in the DMD gene. Their pathophysiological substrate is the defective expression of dystrophin in many tissues. While patients from the same pedigree usually present similar dystrophin expression and clinical course, the extent of cardiac and skeletal muscle involvement may not correlate in the same individual.

Fish oil attenuated dystrophic muscle markers of inflammation via FFA1 and FFA4 in the mdx mouse model of DMD.

In the present study we investigated the involvement of free fatty acid (FFA) receptors in the anti-inflammatory role of eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) in dystrophic muscles, by administering FFA blockers in the mdx mouse model of dystrophy. Mdx mice (3 months-old) were treated with fish oil capsules (FDC Vitamins; 0.4 g EPA and 0.

Checking the shape and lobation of the right atrial appendage in view of their clinical relevance.

Clinically, anatomy of the appendage of the atrium is associated with atrial fibrillation, with the shape and lobation of the appendage having been used to stratify the risk of thromboembolic events. The aim of this study was to examine the age-dependent change in the shape and lobation of the right atrial appendage. A cross-sectional evaluation of the heart of 172 adults and 61 children, fixed in 4% formalin solution was performed.

Identification of plasma interleukins as biomarkers for deflazacort and omega-3 based Duchenne muscular dystrophy therapy.

Duchenne muscular dystrophy (DMD) is a progressive and fatal disease, characterized by the absence of dystrophin, muscle degeneration and cardiorespiratory failure. Creatine kinase is the classic marker to screen for DMD. However, other markers are needed to follow disease progression and to evaluate the response to therapy over longer periods.

Effects of dietary omega-3 on dystrophic cardiac and diaphragm muscles as evaluated by H magnetic resonance spectroscopy: Metabolic profile and calcium-related proteins.

Background & Aims: Duchenne muscular dystrophy (DMD) is characterized by the absence of dystrophin and muscle degeneration. Calcium dysregulation and oxidative stress also contribute to the disease progression. We evaluated the potential therapeutic benefits of supplementation with omega-3 on the metabolic profile, calcium-related proteins and oxidative stress response in the heart and diaphragm (DIA) of the mdx mouse model of DMD at later stages of the disease (13 months).

Dual Therapy Deflazacort/Doxycyclyne Is Better Than Deflazacort Monotherapy to Alleviate Cardiomyopathy in Dystrophin-Deficient mdx Mice.

Cardiomyopathy related to the absence of dystrophin is an important feature in Duchenne muscular dystrophy (DMD) and in the mdx mouse. Doxycycline (DOX) could be a potential therapy for mdx skeletal muscles dystrophy. We investigated whether the corticoid deflazacort (DFZ) plus DOX could improve cardiac mdx dystrophy better than DFZ alone, later (17 months) in dystrophy.

Effects of omega-3 on matrix metalloproteinase-9, myoblast transplantation and satellite cell activation in dystrophin-deficient muscle fibers.

In Duchenne muscular dystrophy (DMD), lack of dystrophin leads to progressive muscle degeneration, with DMD patients suffering from cardiorespiratory failure. Cell therapy is an alternative to life-long corticoid therapy. Satellite cells, the stem cells of skeletal muscles, do not completely compensate for the muscle damage in dystrophic muscles.

P2Y purinergic receptors are highly expressed in cardiac and diaphragm muscles of mdx mice, and their expression is decreased by suramin.

Introduction: In Duchenne muscular dystrophy (DMD) and in the mdx mouse model of DMD, the lack of dystrophin leads to increased calcium influx and muscle necrosis. Patients suffer progressive muscle loss, and cardiomyopathy is an important determinant of morbidity. P2 purinergic receptors participate in the increased calcium levels in dystrophic skeletal muscles.

Effects of fish oil containing eicosapentaenoic acid and docosahexaenoic acid on dystrophic mdx mice hearts at later stages of dystrophy.

Objective: In the present study, we investigated whether omega-3 would be effective against dystrophic cardiomyopathy at later stages (13 and 17 mo) of the disease.

Methods: Mdx mice (8 mo old) received omega-3 oil (commercially available fish oil; FDC vitamins; omega-3) for 5 mo. Untreated-mdx mice received mineral oil.

Diaphragm degeneration and cardiac structure in mdx mouse: potential clinical implications for Duchenne muscular dystrophy.

We examined the effects of exercise on diaphragm degeneration and cardiomyopathy in dystrophin-deficient mdx mice. Mdx mice (11 months of age) were exercised (swimming) for 2 months to worsen diaphragm degeneration. Control mdx mice were kept sedentary.

Changes in calsequestrin, TNF-α, TGF-β and MyoD levels during the progression of skeletal muscle dystrophy in mdx mice: a comparative analysis of the quadriceps, diaphragm and intrinsic laryngeal muscles.

In Duchenne muscular dystrophy (DMD), the search for new biomarkers to follow the evolution of the disease is of fundamental importance in the light of the evolving gene and pharmacological therapies. In addition to the lack of dystrophin, secondary events including changes in calcium levels, inflammation and fibrosis greatly contribute to DMD progression and the molecules involved in these events may represent potential biomarkers. In this study, we performed a comparative evaluation of the progression of dystrophy within muscles that are differently affected by dystrophy (diaphragm; DIA and quadriceps; QDR) or spared (intrinsic laryngeal muscles) using the mdx mice model of DMD.

Expression of calcium-buffering proteins in rat intrinsic laryngeal muscles.

Intrinsic laryngeal muscles (ILM) are highly specialized muscles involved in phonation and airway protection, with unique properties that allow them to perform extremely rapid contractions and to escape from damage in muscle dystrophy. Due to that, they may differ from limb muscles in several physiological aspects. Because a better ability to handle intracellular calcium has been suggested to explain ILM unique properties, we hypothesized that the profile of the proteins that regulate calcium levels in ILM is different from that in a limb muscle.

Long-Term Therapy With Omega-3 Ameliorates Myonecrosis and Benefits Skeletal Muscle Regeneration in Mdx Mice.

In Duchenne muscle dystrophy (DMD) and in the mdx mouse model of DMD, a lack of dystrophin leads to myonecrosis and cardiorespiratory failure. Several lines of evidence suggest a detrimental role of the inflammatory process in the dystrophic process. Previously, we demonstrated that short-term therapy with eicosapentaenoic acid (EPA), at early stages of disease, ameliorated dystrophy progression in the mdx mouse.

Co-administration of deflazacort and doxycycline: a potential pharmacotherapy for Duchenne muscular dystrophy.

The standard therapy used in the treatment of Duchenne muscle dystrophy (DMD) is corticoids, such as deflazacort and prednisone. However, they have limited therapeutic value, and their combination with drugs already in use to treat other human diseases could potentially increase corticoid outcomes in DMD. In the present study, we evaluated whether a combined therapy of the corticoid deflazacort with doxycycline could result in greater improvement in mdx dystrophy than deflazacort alone.

Effect of N-acetylcysteine plus deferoxamine on oxidative stress and inflammation in dystrophic muscle cells.

Objectives: Oxidative stress and inflammatory process play an important role in the pathogenesis of Duchenne muscular dystrophy (DMD). We investigated whether deferoxamine (DFX) improves the antioxidant effects of N-acetylcysteine (NAC) on primary cultures of dystrophic muscle cells from mdx mice, the experimental model of DMD.

Methods: Primary cultures of skeletal muscle cells from mdx mice were treated with either NAC (10 mM), DFX (5 mM), or NAC plus DFX for 24 hours.

Understanding the beneficial effects of doxycycline on the dystrophic phenotype of the mdx mouse.

Introduction: The purpose of this study was to better understand the beneficial effects of doxycycline on the dystrophic muscles of the mdx mouse.

Methods: Doxycycline (DOX) was administered for 36 days, starting on postnatal day 0, via drinking water. Untreated mdx mice received plain water for the same period and served as a control group.

EPA protects against muscle damage in the mdx mouse model of Duchenne muscular dystrophy by promoting a shift from the M1 to M2 macrophage phenotype.

In dystrophic mdx mice and in Duchenne muscular dystrophy, inflammation contributes to myonecrosis. Previously, we demonstrated that eicosapentaenoic acid (EPA) decreased inflammation and necrosis in dystrophic muscle. In the present study, we examined the effects of EPA and the corticoid deflazacort (DFZ) as modulators of M1 (iNOS-expressing cells) and M2 (CD206-expressing cells) macrophages.

Isobaric Tagging-Based Quantification for Proteomic Analysis: A Comparative Study of Spared and Affected Muscles from mdx Mice at the Early Phase of Dystrophy.

Duchenne muscular dystrophy (DMD) is the most common childhood myopathy, characterized by muscle loss and cardiorespiratory failure. While the genetic basis of DMD is well established, secondary mechanisms associated with dystrophic pathophysiology are not fully clarified yet. In order to obtain new insights into the molecular mechanisms of muscle dystrophy during earlier stages of the disease, we performed a comparative proteomic profile of the spared extraocular muscles (EOM) vs.

Suramin attenuates dystrophin-deficient cardiomyopathy in the mdx mouse model of duchenne muscular dystrophy.

Introduction: The purpose of this study was to determine the effects of suramin, an antifibrotic agent, on cardiac function and remodeling in mdx mice.

Methods: mdx mice (8 months old) received intraperitoneal injections of suramin twice a week for 3 months. Control mdx mice (8 months old) were injected with saline.

Effects of fish oil containing eicosapentaenoic acid and docosahexaenoic acid on dystrophic mdx mice.

Background & Aims: In Duchenne muscular dystrophy (DMD) and in the mdx mouse model of DMD, the lack of dystrophin leads to muscle degeneration and inflammation contributes to progression of the disease. In this study, we evaluated the effects of commercially available fish oil containing EPA and docosahexaenoic acid (DHA) on mdx.

Methods: Mdx mice (14 days old) were treated with fish oil (FDC Vitamins; 0.

Suramin affects metalloproteinase-9 activity and increases β-dystroglycan levels in the diaphragm of the dystrophin-deficient mdx mouse.

Introduction: In Duchenne muscular dystrophy and in the mdx mouse, muscle fiber degeneration and subsequent fibrosis lead to cardiorespiratory failure. Previously, we demonstrated that the anti-fibrotic agent suramin was effective in decreasing fibrosis in mdx muscles. In this study, we were interested to see whether suramin could affect metalloproteinases (MMP) and improve the functional activity of the mdx diaphragm muscle.