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muscular protein synthesis

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https://www.readbyqxmd.com/read/29134705/a-dystroglycan-mutation-p-cys667phe-associated-to-muscle-eye-brain-disease-with-multicystic-leucodystrophy-results-in-er-retention-of-the-mutant-protein
#1
Giulia Signorino, Sonia Covaceuszach, Manuela Bozzi, Wolfgang Hubner, Viola Mönkemöller, Petr V Konarev, Alberto Cassetta, Andrea Brancaccio, Francesca Sciandra
Dystroglycan (DG) is a cell adhesion complex composed by two subunits, the highly glycosylated α-DG and the transmembrane β-DG. In skeletal muscle, DG is involved in dystroglycanopathies, a group of heterogeneous muscular dystrophies characterized by a reduced glycosylation of α-DG. The genes mutated in secondary dystroglycanopathies are involved in the synthesis of O-mannosyl glycans and in the O-mannosylation pathway of α-DG. Mutations in the DG gene (DAG1), causing primary dystroglycanopathies, destabilize the α-DG core protein influencing its binding to modifying enzymes...
November 13, 2017: Human Mutation
https://www.readbyqxmd.com/read/29122965/the-impact-of-dairy-protein-during-limb-immobilization-and-recovery-on-muscle-size-and-protein-synthesis-a-randomized-controlled-trial
#2
Cameron J Mitchell, Randall F D'Souza, Sarah M Mitchell, Vandre Casagrande Figueiredo, Benjamin F Miller, Karyn L Hamilton, Frederick F Peelor, Marcelli Coronet, Chantal A Pileggi, Brenan Durainayagam, Aaron C Fanning, Sally D Poppitt, David Cameron-Smith
Muscle disuse results in the loss of muscular strength and size, due to an imbalance between protein synthesis (MPS) and breakdown (MPB). Protein ingestion stimulates MPS, although it is not established if protein is able to attenuate muscle loss with immobilization (IM) or influence the recovery consisting of ambulatory movement followed by resistance training (RT). Thirty men (49.9±0.6 years) underwent 14 days of unilateral leg IM, 14 days of ambulatory recovery (AR) and a further 6 RT sessions over 14 days...
November 9, 2017: Journal of Applied Physiology
https://www.readbyqxmd.com/read/29099523/a-potential-strategy-for-counteracting-age-related-sarcopenia-preliminary-evidence-of-combined-exercise-training-and-leucine-supplementation
#3
Z Xia, J M Cholewa, Y Zhao, Y Yang, H Shang, H Jiang, Q Su, N E Zanchi
Previous research has demonstrated the positive effects of concurrent/combined aerobic and resistance exercise or leucine supplementation on skeletal muscle protein synthesis (MPS) and hypertrophy in aging organisms. However, the effects of a multimodal intervention which combines both aerobic and resistance exercise and leucine supplementation has not been fully elucidated. Eighteen month old and 2 month old C57BL/6 mice were assigned to aging control (AC, n = 8), aging and multimodal intervention (AMI, n = 8) and young control (YC, n = 8)...
November 3, 2017: Food & Function
https://www.readbyqxmd.com/read/29095865/beneficial-effects-of-high-dose-taurine-treatment-in-juvenile-dystrophic-mdx-mice-are-offset-by-growth-restriction
#4
Jessica R Terrill, Gavin J Pinniger, Keshav V Nair, Miranda D Grounds, Peter G Arthur
Duchenne Muscular Dystrophy (DMD) is a fatal muscle wasting disease manifested in young boys, for which there is no current cure. We have shown that the amino acid taurine is safe and effective at preventing dystropathology in the mdx mouse model for DMD. This study aimed to establish if treating growing mdx mice with a higher dose of taurine was more effective at improving strength and reducing inflammation and oxidative stress. Mice were treated with a dose of taurine estimated to be 16 g/kg/day, in drinking water from 1-6 weeks of age, after which in vivo and ex vivo muscle strength was assessed, as were measures of inflammation, oxidative stress and taurine metabolism...
2017: PloS One
https://www.readbyqxmd.com/read/29081423/muscular-dystrophy-with-ribitol-phosphate-deficiency-a-novel-post-translational-mechanism-in-dystroglycanopathy
#5
Motoi Kanagawa, Tatsushi Toda
Muscular dystrophy is a group of genetic disorders characterized by progressive muscle weakness. In the early 2000s, a new classification of muscular dystrophy, dystroglycanopathy, was established. Dystroglycanopathy often associates with abnormalities in the central nervous system. Currently, at least eighteen genes have been identified that are responsible for dystroglycanopathy, and despite its genetic heterogeneity, its common biochemical feature is abnormal glycosylation of alpha-dystroglycan. Abnormal glycosylation of alpha-dystroglycan reduces its binding activities to ligand proteins, including laminins...
October 24, 2017: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/28954428/ferulic-acid-promotes-hypertrophic-growth-of-fast-skeletal-muscle-in-zebrafish-model
#6
Ya Wen, Hideki Ushio
As a widely distributed and natural existing antioxidant, ferulic acid and its functions have been extensively studied in recent decades. In the present study, hypertrophic growth of fast skeletal myofibers was observed in adult zebrafish after ferulic acid administration for 30 days, being reflected in increased body weight, body mass index (BMI), and muscle mass, along with an enlarged cross-sectional area of myofibers. qRT-PCR analyses demonstrated the up-regulation of relative mRNA expression levels of myogenic transcriptional factors (MyoD, myogenin and serum response factor (SRF)) and their target genes encoding sarcomeric unit proteins involved in muscular hypertrophy (skeletal alpha-actin, myosin heavy chain, tropomyosin, and troponin I)...
September 26, 2017: Nutrients
https://www.readbyqxmd.com/read/28951602/chemotherapeutics-overcoming-nonsense-mutation-associated-genetic-diseases-medicinal-chemistry-of-negamycin
#7
REVIEW
Akihiro Taguchi, Keisuke Hamada, Yoshio Hayashi
Nonsense mutations caused by the presence of an in-frame premature termination codon (PTC) account for ~10% of gene lesions that together cause over 1800 inherited human diseases. One approach to treating genetic diseases that stem from PTCs is selective promotion of translational readthrough in a PTC using 'readthrough compounds' that can lead to partial restoration of full-length functional protein expression. (+)-Negamycin, a natural dipeptide-like antibiotic, may restore some dystrophin expression in the skeletal muscles of mice with Duchenne muscular dystrophy, and this compound has been recognized as a potential therapeutic agent for diseases caused by nonsense mutations...
September 27, 2017: Journal of Antibiotics
https://www.readbyqxmd.com/read/28898813/insight-into-the-modulation-of-intestinal-proteome-of-juvenile-common-carp-cyprinus-carpio-l-after-dietary-exposure-to-zno-nanoparticles
#8
Latifeh Chupani, Hamid Niksirat, Vanessa Lünsmann, Sven-Bastiaan Haange, Martin von Bergen, Nico Jehmlich, Eliska Zuskova
ZnO nanoparticles (NPs) are widely used in industrial and consumer products. Therefore understanding their interaction with biological systems is key to their safe application. Proteomics was applied to assess the sub-lethal effects of dietary ZnO NPs on two parts of carp intestine, the intestinal folds and the muscular parts. A commercial carp feed containing 500mgkg(-1) of ZnO NPs was fed to fish for six weeks. The abundances of 32 proteins in the treated intestinal folds were significantly changed and in addition, 28 proteins were significantly changed in the muscular parts...
February 1, 2018: Science of the Total Environment
https://www.readbyqxmd.com/read/28814401/effects-of-protein-supplementation-combined-with-resistance-exercise-on-body-composition-and-physical-function-in-older-adults-a-systematic-review-and-meta-analysis
#9
REVIEW
Chun-De Liao, Jau-Yih Tsauo, Yen-Tzu Wu, Chin-Pao Cheng, Hui-Chuen Chen, Yi-Ching Huang, Hung-Chou Chen, Tsan-Hon Liou
Background: Overweight and obese older people face a high risk of muscle loss and impaired physical function, which may contribute to sarcopenic obesity. Resistance exercise training (RET) has a beneficial effect on muscle protein synthesis and can be augmented by protein supplementation (PS). However, whether body weight affects the augmentation of muscular and functional performance in response to PS in older people undergoing RET remains unclear.Objective: This study was conducted to identify the effects of PS on the body composition and physical function of older people undergoing RET...
October 2017: American Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/28802074/altered-amino-acid-concentrations-in-nafld-impact-of-obesity-and-insulin-resistance
#10
Melania Gaggini, Fabrizia Carli, Chiara Rosso, Emma Buzzigoli, Milena Marietti, Veronica Della Latta, Demetrio Ciociaro, Maria Lorena Abate, Roberto Gambino, Maurizio Cassader, Elisabetta Bugianesi, Amalia Gastaldelli
Plasma concentrations of amino acids (AA), in particular branched chain (BCAA), are often found increased in non-alcoholic fatty liver disease (NAFLD). However, if this is due to increased muscular protein catabolism, obesity and/or increased insulin resistance (IR) or impaired tissue metabolism is not known. Thus, we evaluated a) if subjects with NAFLD non-obese (NAFLD-NO), compared to obese (NAFLD-Ob) display altered plasma AA compared to controls (CT); b) if AA concentrations are associated to IR and liver histology...
August 12, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28725197/skeletal-muscle-remodeling-in-response-to-eccentric-vs-concentric-loading-morphological-molecular-and-metabolic-adaptations
#11
REVIEW
Martino V Franchi, Neil D Reeves, Marco V Narici
Skeletal muscle contracts either by shortening or lengthening (concentrically or eccentrically, respectively); however, the two contractions substantially differ from one another in terms of mechanisms of force generation, maximum force production and energy cost. It is generally known that eccentric actions generate greater force than isometric and concentric contractions and at a lower metabolic cost. Hence, by virtue of the greater mechanical loading involved in active lengthening, eccentric resistance training (ECC RT) is assumed to produce greater hypertrophy than concentric resistance training (CON RT)...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28690550/targeting-inflammation-and-downstream-protein-metabolism-in-sarcopenia-a-brief-up-dated-description-of-concurrent-exercise-and-leucine-based-multimodal-intervention
#12
REVIEW
Zhi Xia, Jason Cholewa, Yan Zhao, Hua-Yu Shang, Yue-Qin Yang, Kassiana Araújo Pessôa, Quan-Sheng Su, Fernanda Lima-Soares, Nelo Eidy Zanchi
Sarcopenia is defined as the progressive loss of muscle mass with age, and poses a serious threat to the physiological and psychological health of the elderly population with consequential economic and social burdens. Chronic low-grade inflammation plays a central role in the development of sarcopenia such that it alters cellular protein metabolism to favor proteolysis over synthesis, and thereby accelerates muscular atrophy. The purpose of this review is to highlight how exercise and nutrition intervention strategies can attenuate or treat sarcopenia...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28683291/lack-of-glycogenin-causes-glycogen-accumulation-and-muscle-function-impairment
#13
Giorgia Testoni, Jordi Duran, Mar García-Rocha, Francisco Vilaplana, Antonio L Serrano, David Sebastián, Iliana López-Soldado, Mitchell A Sullivan, Felipe Slebe, Marta Vilaseca, Pura Muñoz-Cánoves, Joan J Guinovart
Glycogenin is considered essential for glycogen synthesis, as it acts as a primer for the initiation of the polysaccharide chain. Against expectations, glycogenin-deficient mice (Gyg KO) accumulate high amounts of glycogen in striated muscle. Furthermore, this glycogen contains no covalently bound protein, thereby demonstrating that a protein primer is not strictly necessary for the synthesis of the polysaccharide in vivo. Strikingly, in spite of the higher glycogen content, Gyg KO mice showed lower resting energy expenditure and less resistance than control animals when subjected to endurance exercise...
July 5, 2017: Cell Metabolism
https://www.readbyqxmd.com/read/28653137/duchenne-muscular-dystrophy-a-practice-update
#14
Renu Suthar, Naveen Sankhyan
Duchenne Muscular Dystrophy (DMD) is an X-linked recessive disorder caused by a deficient or defective synthesis of dystrophin protein. DMD is the most common form of muscular dystrophy with an incidence of about 1 in 5000 live boys. Though primarily resulting in progressive muscle weakness, it affects various other organs as well. Heart, brain and smooth muscles are commonly involved, because of expression of dystrophin in these organs. The management of DMD requires a multidisciplinary liaison, anticipatory management and prevention of the complications...
June 27, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28624227/efficient-smn-rescue-following-subcutaneous-tricyclo-dna-antisense-oligonucleotide-treatment
#15
Valérie Robin, Graziella Griffith, John-Paul L Carter, Christian J Leumann, Luis Garcia, Aurélie Goyenvalle
Spinal muscular atrophy (SMA) is a recessive disease caused by mutations in the SMN1 gene, which encodes the protein survival motor neuron (SMN), whose absence dramatically affects the survival of motor neurons. In humans, the severity of the disease is lessened by the presence of a gene copy, SMN2. SMN2 differs from SMN1 by a C-to-T transition in exon 7, which modifies pre-mRNA splicing and prevents successful SMN synthesis. Splice-switching approaches using antisense oligonucleotides (AONs) have already been shown to correct this SMN2 gene transition, providing a therapeutic avenue for SMA...
June 16, 2017: Molecular Therapy. Nucleic Acids
https://www.readbyqxmd.com/read/28623080/increased-constitutive-nitric-oxide-production-by-whole-body-periodic-acceleration-ameliorates-alterations-in-cardiomyocytes-associated-with-utrophin-dystrophin-deficiency
#16
Jose R Lopez, Juan Kolster, Rui Zhang, Jose Adams
Duchenne Muscular Dystrophy (DMD) cardiomyopathy is a progressive lethal disease caused by the lack of the dystrophin protein in the heart. The most widely used animal model of DMD is the dystrophin-deficient mdx mouse; however, these mice exhibit a mild dystrophic phenotype with heart failure only late in life. In contrast, mice deficient for both dystrophin and utrophin (mdx/utrn(-/-), or dKO) can be used to model severe DMD cardiomyopathy where pathophysiological indicators of heart failure are detectable by 8-10weeks of age...
June 13, 2017: Journal of Molecular and Cellular Cardiology
https://www.readbyqxmd.com/read/28607086/specific-targeting-of-tgf-%C3%AE-family-ligands-demonstrates-distinct-roles-in-the-regulation-of-muscle-mass-in-health-and-disease
#17
Justin L Chen, Kelly L Walton, Adam Hagg, Timothy D Colgan, Katharine Johnson, Hongwei Qian, Paul Gregorevic, Craig A Harrison
The transforming growth factor-β (TGF-β) network of ligands and intracellular signaling proteins is a subject of intense interest within the field of skeletal muscle biology. To define the relative contribution of endogenous TGF-β proteins to the negative regulation of muscle mass via their activation of the Smad2/3 signaling axis, we used local injection of adeno-associated viral vectors (AAVs) encoding ligand-specific antagonists into the tibialis anterior (TA) muscles of C57BL/6 mice. Eight weeks after AAV injection, inhibition of activin A and activin B signaling produced moderate (∼20%), but significant, increases in TA mass, indicating that endogenous activins repress muscle growth...
June 27, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28594869/compound-heterozygous-mutations-in-glycyl-trna-synthetase-gars-cause-mitochondrial-respiratory-chain-dysfunction
#18
Michael Nafisinia, Lisa G Riley, Wendy A Gold, Kaustuv Bhattacharya, Carolyn R Broderick, David R Thorburn, Cas Simons, John Christodoulou
Glycyl-tRNA synthetase (GARS; OMIM 600287) is one of thirty-seven tRNA-synthetase genes that catalyses the synthesis of glycyl-tRNA, which is required to insert glycine into proteins within the cytosol and mitochondria. To date, eighteen mutations in GARS have been reported in patients with autosomal-dominant Charcot-Marie-Tooth disease type 2D (CMT2D; OMIM 601472), and/or distal spinal muscular atrophy type V (dSMA-V; OMIM 600794). In this study, we report a patient with clinical and biochemical features suggestive of a mitochondrial respiratory chain (MRC) disorder including mild left ventricular posterior wall hypertrophy, exercise intolerance, and lactic acidosis...
2017: PloS One
https://www.readbyqxmd.com/read/28588499/intake-of-a-ketone-ester-drink-during-recovery-from-exercise-promotes-mtorc1-signaling-but-not-glycogen-resynthesis-in-human-muscle
#19
Tijs Vandoorne, Stefan De Smet, Monique Ramaekers, Ruud Van Thienen, Katrien De Bock, Kieran Clarke, Peter Hespel
Purpose: Ketone bodies are energy substrates produced by the liver during prolonged fasting or low-carbohydrate diet. The ingestion of a ketone ester (KE) rapidly increases blood ketone levels independent of nutritional status. KE has recently been shown to improve exercise performance, but whether it can also promote post-exercise muscle protein or glycogen synthesis is unknown. Methods: Eight healthy trained males participated in a randomized double-blind placebo-controlled crossover study. In each session, subjects undertook a bout of intense one-leg glycogen-depleting exercise followed by a 5-h recovery period during which they ingested a protein/carbohydrate mixture...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28546288/dynamic-changes-in-the-mouse-skeletal-muscle-proteome-during-denervation-induced-atrophy
#20
Franziska Lang, Sriram Aravamudhan, Hendrik Nolte, Clara Türk, Soraya Hölper, Stefan Müller, Stefan Günther, Bert Blaauw, Thomas Braun, Marcus Krüger
Loss of neuronal stimulation enhances protein breakdown and reduces protein synthesis, causing rapid loss of muscle mass. To elucidate the pathophysiological adaptations that occur in atrophying muscles, we used stable isotope labelling and mass spectrometry to quantify protein expression changes accurately during denervation-induced atrophy after sciatic nerve section in the mouse gastrocnemius muscle. Additionally, mice were fed a stable isotope labelling of amino acids in cell culture (SILAC) diet containing (13)C6-lysine for 4, 7 or 11 days to calculate relative levels of protein synthesis in denervated and control muscles...
July 1, 2017: Disease Models & Mechanisms
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