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Total 180 results found since Jan 2013.

Distal Arthrogryposis and Lethal Congenital Contracture Syndrome – An Overview
Distal arthrogryposis (DA) is a skeletal muscle disorder which can be classified under a broader term as Arthrogryposis multiplex contractures. DA is characterized by the presence of joint contractures at various parts of the body, particularly in distal extremities. It is identified as an autosomal dominant and a rare X-linked recessive disorder associated with increased connective tissue formation around joints in such way that immobilizes muscle movement causing deformities. DA is again classified into various types since it manifests as a range of conditions representing different etiologies. Myopathy is one of the mos...
Source: Frontiers in Physiology - June 24, 2020 Category: Physiology Source Type: research

Physiological and Pathological Mitochondrial Clearance Is Related to Pectoralis Major Muscle Pathogenesis in Broilers With Wooden Breast Syndrome
In this study, we focused on mitochondrial morphology and dynamics in the myofibers, as these organelles are sensitive to damage by hypoxia, and examined the effects on WB pathogenesis. Specifically, the PMs of a flock of 35 broilers at 50 days of age were evaluated. First, the severity of disease in each bird was determined by measuring histopathological indices including the fibrotic area (FA) in the muscle and circularity of myofibers (CM). These values were 29.4 ± 9.6% and 0.70 ± 0.042, respectively, showing variety among the flock. Myofiber vacuolization was observed in all birds including numerous small- or large-r...
Source: Frontiers in Physiology - June 15, 2020 Category: Physiology Source Type: research

Muscle Abnormalities and Meat Quality Consequences in Modern Turkey Hybrids
Turkey meat is the second most consumed poultry meat worldwide and represents an economic source of high-quality protein for human consumption. To fulfill the increasing demand for turkey meat, breeding companies have been selecting genetic lines with increased growth potential and breast muscle proportion. Moreover, the progressive shift toward further processed products has emphasized the need for higher standards in poultry meat to improve its technological characteristics and functional properties (i.e., water-holding capacity). However, as observed for broiler chickens, a growing body of scientific evidence suggests t...
Source: Frontiers in Physiology - June 11, 2020 Category: Physiology Source Type: research

Aerobic Exercise Training in Patients With mtDNA-Related Mitochondrial Myopathy
In patients with mitochondrial DNA (mtDNA) mutation, a pathogenic mtDNA mutation is heteroplasmically distributed among tissues. The ratio between wild-type and mutated mtDNA copies determines the mtDNA mutation load of the tissue, which correlates inversively with oxidative capacity of the tissue. In patients with mtDNA mutation, the mutation load is often very high in skeletal muscle compared to other tissues. Additionally, skeletal muscle can increase its oxygen demand up to 100-fold from rest to exercise, which is unmatched by any other tissue. Thus, exercise intolerance is the most common symptom in patients with mtDN...
Source: Frontiers in Physiology - May 20, 2020 Category: Physiology Source Type: research

Pectoralis Major (Breast) Muscle Extracellular Matrix Fibrillar Collagen Modifications Associated With the Wooden Breast Fibrotic Myopathy in Broilers
The poultry industry has placed significant emphasis on the selection of meat-type broilers for increased body weight, increased meat yield especially the pectoralis major (breast) muscle, decreased time to processing, and improved feed conversion. Although significant improvements have occurred in fast-growing meat-type broilers, myopathies affecting meat quality especially in the pectoralis major muscle have occurred. Many of the broiler breast muscle myopathies are caused by inflammation leading to the necrosis of existing muscle fibers and resulting in replacement of the muscle fibers with extracellular matrix proteins...
Source: Frontiers in Physiology - May 6, 2020 Category: Physiology Source Type: research

Altered Sarcomeric Structure and Function in Woody Breast Myopathy of Avian Pectoralis Major Muscle
In conclusion, the results show that the rapid growth of muscle in woody breast disease is associated with significant structural and functional changes in the pectoralis major musculature, associated with alterations in the mechanical anchoring of contractile filaments.
Source: Frontiers in Physiology - April 8, 2020 Category: Physiology Source Type: research

Blood Gas Disturbances and Disproportionate Body Weight Distribution in Broilers With Wooden Breast
Wooden breast syndrome is a widespread and economically important myopathy and vasculopathy of fast growing, commercial broiler chickens, primarily affecting birds with high feed efficiency and large breast muscle yield. To investigate potential systemic physiological differences between birds affected and unaffected by wooden breast, a total of 103 market-age Cobb 500 broilers were sampled for 13 blood parameters and the relative weights of the pectoralis major muscle, pectoralis minor muscle, external oblique muscle, wing, heart, lungs, liver, and spleen. Blood analysis was performed on samples taken from the brachial ve...
Source: Frontiers in Physiology - April 6, 2020 Category: Physiology Source Type: research

Skeletal muscle CaV1.1 channelopathies.
Abstract CaV1.1 is specifically expressed in skeletal muscle where it functions as voltage sensor of skeletal muscle excitation-contraction (EC) coupling independently of its functions as L-type calcium channel. Consequently, all known CaV1.1-related diseases are muscle diseases and the molecular and cellular disease mechanisms relate to the dual functions of CaV1.1 in this tissue. To date, four types of muscle diseases are known that can be linked to mutations in the CACNA1S gene or to splicing defects. These are hypo- and normokalemic periodic paralysis, malignant hyperthermia susceptibility, CaV1.1-related myop...
Source: Pflugers Archiv : European Journal of Physiology - March 27, 2020 Category: Physiology Authors: Flucher BE Tags: Pflugers Arch Source Type: research

Evaluation via Supervised Machine Learning of the Broiler Pectoralis Major and Liver Transcriptome in Association With the Muscle Myopathy Wooden Breast
The muscle myopathy wooden breast (WB) has recently appeared in broiler production and has a negative impact on meat quality. WB is described as hard/firm consistency found within the pectoralis major (PM). In the present study, we use machine learning from our PM and liver transcriptome dataset to capture the complex relationships that are not typically revealed by traditional statistical methods. Gene expression data was evaluated between the PM and liver of birds with WB and those that were normal. Two separate machine learning algorithms were performed to analyze the data set including the sequential minimal optimizati...
Source: Frontiers in Physiology - February 24, 2020 Category: Physiology Source Type: research

A Critical Appraisal of the Tafazzin Knockdown Mouse Model of Barth Syndrome: What Have We Learned About Pathogenesis and Potential Treatments?
Abstract Pediatric heart failure remains poorly understood, distinct in many aspects from adult heart failure. Limited data point to roles of altered mitochondrial functioning and in particular, changes in mitochondrial lipids, especially cardiolipin. Barth syndrome is a mitochondrial disorder caused by tafazzin mutations that lead to abnormal cardiolipin profiles. Patients are afflicted by cardiomyopathy, skeletal myopathy, neutropenia, and growth delay. A mouse model of Barth syndrome was developed a decade ago, which relies on a doxycycline-inducible shRNA to knock down expression of tafazzin mRNA ("TAZKD"). Ou...
Source: American Journal of Physiology. Heart and Circulatory Physiology - October 10, 2019 Category: Physiology Authors: Ren M, Miller PC, Schlame M, Phoon CKL Tags: Am J Physiol Heart Circ Physiol Source Type: research

Hypoxia inducible factor-prolyl hydroxylase inhibitor ameliorates the myopathy in a mice model of chronic kidney disease.
Abstract Muscle wasting and diminished physical performance contribute to the morbidity and mortality of chronic kidney disease (CKD), for which no curative therapy exists. Accumulating evidence indicates that impaired angiogenesis occurs in the muscles of CKD models. Pro-angiogenesis therapy is therefore considered a potentially effective strategy for limiting CKD-associated myopathy. The hypoxia inducible factor-prolyl hydroxylase inhibitor (HIF-PHI) stabilizes HIF and enhances muscle angiogenesis during acute ischemia, however, little evidence was available from CKD models. Here, we assessed whether the pharmac...
Source: American Journal of Physiology. Renal Physiology - October 6, 2019 Category: Physiology Authors: Qian FY, Li ZL, Guo YD, Gao HC, Gu LH, Le K, Xie CM, Wang B, Zhang ZJ Tags: Am J Physiol Renal Physiol Source Type: research

Quantum Blue Reduces the Severity of Woody Breast Myopathy via Modulation of Oxygen Homeostasis-Related Genes in Broiler Chickens
In this study, using diffuse reflectance spectroscopy (DRS) coupled with iSTAT portable clinical analyzer, we provide evidence that the circulatory- and breast muscle-oxygen homeostasis is dysregulated [low oxygen and hemoglobin (HB) levels] in chickens with WB myopathy compared to healthy counterparts. Molecular analysis showed that blood HB subunit Mu (HBM), Zeta (HBZ), and hephaestin (HEPH) expression were significantly down regulated; however, the expression of the subunit rho of HB beta (HBBR) was upregulated in chicken with WB compared to healthy counterparts. The breast muscle HBBR, HBE, HBZ, and hypoxia-inducible f...
Source: Frontiers in Physiology - September 30, 2019 Category: Physiology Source Type: research

Temporal Association Between Ischemic Muscle Perfusion Recovery and the Restoration of Muscle Contractile Function After Hindlimb Ischemia
We examined this relationship in BALB/cJ mice (N=24) following unilateral hindlimb ischemia (HLI). Specifically, we examined this relationship in two phenotypically distinct muscles (i.e. ‘oxidative’ soleus - Sol and ‘glycolytic’ extensor digitorum longus - EDL) 14- or 56-days after HLI. Although overall limb blood flow (LDPI) reached its’ recovery peak (48% of control) by HLI d14, the capillary networks in both the Sol and EDL (whole mount confocal imaging) were disrupted and competent muscle capillary perfusion (perfused lectin+ μm2 / muscle μm2) remained reduced. Interestingly, both Sol and EDL muscles recov...
Source: Frontiers in Physiology - June 27, 2019 Category: Physiology Source Type: research

RNA-Seq Analysis Reveals Spatial and Sex Differences in Pectoralis Major Muscle of Broiler Chickens Contributing to Difference in Susceptibility to Wooden Breast Disease
This study aimed to characterize biological differences in the p. major between regions of the muscle and sexes of birds. Samples were taken from the cranial and caudal aspects of p. major muscles of 3-week-old, unaffected male and female birds for RNA sequencing. RNA was extracted using the mirVana miRNA Isolation kit (ThermoScientific), and complimentary DNA (cDNA) libraries were prepared using the TruSeq Stranded mRNA Sample Preparation Kit (Illumina). Libraries were sequenced by the Delaware Biotechnology Institute (DBI) using HiSeq2500. Sequence reads were aligned to the chicken reference genome with HISAT, and genes ...
Source: Frontiers in Physiology - June 17, 2019 Category: Physiology Source Type: research

The loss of slow skeletal muscle isoform of troponin T in spindle intrafusal fibers explains the pathophysiology of Amish nemaline myopathy.
This article is protected by copyright. All rights reserved. PMID: 31148174 [PubMed - as supplied by publisher]
Source: The Journal of Physiology - May 30, 2019 Category: Physiology Authors: Oki K, Wei B, Feng HZ, Jin JP Tags: J Physiol Source Type: research