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https://www.readbyqxmd.com/read/28087734/increased-mitophagy-in-the-skeletal-muscle-of-spinal-and-bulbar-muscular-atrophy-patients
#1
Doriana Borgia, Adriana Malena, Marco Spinazzi, Maria Andrea Desbats, Leonardo Salviati, Aaron P Russell, Giovanni Miotto, Laura Tosatto, Elena Pegoraro, Gianni Sorarù, Maria Pennuto, Lodovica Vergani
Spinal and bulbar muscular atrophy (SBMA) is a neuromuscular disorder caused by polyglutamine expansion in the androgen receptor (AR) and characterized by the loss of lower motor neurons. Here we investigated pathological processes occurring in muscle biopsy specimens derived from SBMA patients and, as controls, age-matched healthy subjects and patients suffering from amyotrophic lateral sclerosis (ALS) and neurogenic atrophy. We detected atrophic fibers in the muscle of SBMA, ALS and neurogenic atrophy patients...
January 13, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28069130/comparison-of-gel-properties-and-biochemical-characteristics-of-myofibrillar-protein-from-bighead-carp-aristichthys-nobilis-affected-by-frozen-storage-and-a-hydroxyl-radical-generation-oxidizing-system
#2
Han Lu, Longteng Zhang, Qingzheng Li, Yongkang Luo
We wanted to clarify whether gel properties can be affected by in vivo or in vitro myofibrillar protein oxidation and, thus, to provide relevant information and a scientific foundation for the processing of gel products. To accomplish this, we measured the changes in sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE), total disulfide (SS) content, surface hydrophobicity (So-ANS), carbonyl content, and gel texture and water-holding capacity (WHC) of isolated myofibrillar protein from bighead carp fillets during frozen storage and under different H2O2 concentrations, which were used to represent in vivo and in vitro conditions, respectively...
May 15, 2017: Food Chemistry
https://www.readbyqxmd.com/read/28064564/aspartate-inhibits-lps-induced-mafbx-and-murf1-expression-in-skeletal-muscle-in-weaned-pigs-by-regulating-akt-ampk%C3%AE-and-foxo1
#3
Yulan Liu, Xiuying Wang, Weibo Leng, Dingan Pi, Zhixiao Tu, Huiling Zhu, Haifeng Shi, Shuang Li, Yongqing Hou, Chien-An Andy Hu
Infection and inflammation can result in the rapid loss of muscle mass and myofibrillar proteins (muscle atrophy). In addition, aspartate (Asp) is necessary for protein synthesis in mammalian cells. We hypothesized that Asp could attenuate LPS-induced muscle atrophy in a piglet model. Twenty-four weaning piglets were allotted to four treatments, including non-challenged control, LPS challenged control, LPS+0.5% Asp and LPS+1.0% Asp. On d 21, the piglets were injected with i.p. injection of LPS (100 ug/kg BM) or saline...
January 2017: Innate Immunity
https://www.readbyqxmd.com/read/28057584/coregulator-mediated-control-of-skeletal-muscle-plasticity-a-mini-review
#4
Svenia Schnyder, Barbara Kupr, Christoph Handschin
Skeletal muscle plasticity is a complex process entailing massive transcriptional programs. These changes are mediated by the action of nuclear receptors and other transcription factors. In addition, coregulator proteins have emerged as important players in this process by linking transcription factors to the RNA polymerase II complex and inducing changes in the chromatic structure. An accumulating body of work highlights the pleiotropic functions of coregulator proteins in the control of tissue-specific and whole body metabolism...
January 2, 2017: Biochimie
https://www.readbyqxmd.com/read/28051178/muscle-specific-deletion-of-bdk-amplifies-loss-of-myofibrillar-protein-during-protein-undernutrition
#5
Takuya Ishikawa, Yasuyuki Kitaura, Yoshihiro Kadota, Yukako Morishita, Miki Ota, Fumiya Yamanaka, Minjun Xu, Masahito Ikawa, Naokazu Inoue, Fuminori Kawano, Naoya Nakai, Taro Murakami, Shinji Miura, Yukino Hatazawa, Yasutomi Kamei, Yoshiharu Shimomura
Branched-chain amino acids (BCAAs) are essential amino acids for mammals and play key roles in the regulation of protein metabolism. However, the effect of BCAA deficiency on protein metabolism in skeletal muscle in vivo remains unclear. Here we generated mice with lower BCAA concentrations by specifically accelerating BCAA catabolism in skeletal muscle and heart (BDK-mKO mice). The mice appeared to be healthy without any obvious defects when fed a protein-rich diet; however, bolus ingestion of BCAAs showed that mTORC1 sensitivity in skeletal muscle was enhanced in BDK-mKO mice compared to the corresponding control mice...
January 4, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28040161/evaluation-of-the-spoilage-potential-of-bacteria-isolated-from-chilled-chicken-in%C3%A2-vitro-and-in-situ
#6
Guang-Yu Wang, Hu-Hu Wang, Yi-Wei Han, Tong Xing, Ke-Ping Ye, Xing-Lian Xu, Guang-Hong Zhou
Microorganisms play an important role in the spoilage of chilled chicken. In this study, a total of 53 isolates, belonging to 7 species of 3 genera, were isolated using a selective medium based on the capacity to spoil chicken juice. Four isolates, namely Aeromonas salmonicida 35, Pseudomonas fluorescens H5, Pseudomonas fragi H8 and Serratia liquefaciens 17, were further characterized to assess their proteolytic activities in vitro using meat protein extracts and to evaluate their spoilage potential in situ...
May 2017: Food Microbiology
https://www.readbyqxmd.com/read/28028127/myofibrillar-z-discs-are-a-protein-phosphorylation-hot-spot-with-pkc-%C3%AE-modulating-protein-dynamics
#7
Lena Reimann, Heike Wiese, Yvonne Leber, Anja N Schwäble, Anna L Fricke, Anne Rohland, Bettina Knapp, Christian D Peikert, Friedel Drepper, Peter F M van der Ven, Gerald Radziwill, Dieter O Fürst, Bettina Warscheid
The Z-disc is a protein-rich structure critically important for the development and integrity of myofibrils, which are the contractile organelles of cross-striated muscle cells. We here used mouse C2C12 myoblast which were differentiated into myotubes followed by electrical pulse stimulation (EPS) to generate contracting myotubes comprising mature Z-discs. Using a quantitative proteomics approach, we analyzed changes in the relative abundance of 2,588 proteins, of which 387 were significantly regulated in myoblasts versus myotubes...
December 27, 2016: Molecular & Cellular Proteomics: MCP
https://www.readbyqxmd.com/read/28017980/combining-ozone-and-slurry-ice-to-maximize-shelf-life-and-quality-of-bighead-croaker-collichthys-niveatus
#8
Jing Chen, Ju Huang, Shanggui Deng, Yuting Huang
Slurry ice possesses several advantages for seafood preservation, such as faster chilling rate, reduced physical damage, and complete coverage of the fish surface. In this paper, the efficiency of its employment, either applied alone or combined with ozone, was evaluated for bighead croaker and compared with traditional flake ice. Changes of a series of microbial, biochemical and sensory parameters including total viable count (TVC), sensory scores, total volatile basic nitrogen (TVB-N), peroxide value (PV), thiobarbituric acid (TBA), hardness and springiness were investigated up to 21 days...
October 2016: Journal of Food Science and Technology
https://www.readbyqxmd.com/read/28017449/anabolic-resistance-does-not-explain-sarcopenia-in-patients-with-type-2-diabetes-mellitus-compared-with-healthy-controls-despite-reduced-mtor-pathway-activity
#9
Daniel J Cuthbertson, John Babraj, Graham Leese, Mario Siervo
BACKGROUND: Ageing and type 2 diabetes mellitus (T2DM) are risk factors for skeletal muscle loss. We investigated whether anabolic resistance to feeding might underlie accelerated muscle loss in older people with T2DM and whether dysregulated mTOR signalling was implicated. SUBJECTS: 8 obese men with T2DM, and 12 age-matched controls were studied (age 68 ± 3 vs. 68±6 y; BMI: 30 ± 2 vs. 27 ± 5 kg m(-2)). METHODS: Body composition was measured by dual-X-ray absorptiometry...
November 25, 2016: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/28012042/dihydropyridine-receptor-dhpr-cacna1s-congenital-myopathy
#10
Vanessa Schartner, Norma B Romero, Sandra Donkervoort, Susan Treves, Pinki Munot, Tyler Mark Pierson, Ivana Dabaj, Edoardo Malfatti, Irina T Zaharieva, Francesco Zorzato, Osorio Abath Neto, Guy Brochier, Xavière Lornage, Bruno Eymard, Ana Lía Taratuto, Johann Böhm, Hernan Gonorazky, Leigh Ramos-Platt, Lucy Feng, Rahul Phadke, Diana X Bharucha-Goebel, Charlotte Jane Sumner, Mai Thao Bui, Emmanuelle Lacene, Maud Beuvin, Clémence Labasse, Nicolas Dondaine, Raphael Schneider, Julie Thompson, Anne Boland, Jean-François Deleuze, Emma Matthews, Aleksandra Nadaj Pakleza, Caroline A Sewry, Valérie Biancalana, Susana Quijano-Roy, Francesco Muntoni, Michel Fardeau, Carsten G Bönnemann, Jocelyn Laporte
Muscle contraction upon nerve stimulation relies on excitation-contraction coupling (ECC) to promote the rapid and generalized release of calcium within myofibers. In skeletal muscle, ECC is performed by the direct coupling of a voltage-gated L-type Ca(2+) channel (dihydropyridine receptor; DHPR) located on the T-tubule with a Ca(2+) release channel (ryanodine receptor; RYR1) on the sarcoplasmic reticulum (SR) component of the triad. Here, we characterize a novel class of congenital myopathy at the morphological, molecular, and functional levels...
December 23, 2016: Acta Neuropathologica
https://www.readbyqxmd.com/read/28011132/predominant-processing-adaptability-of-staphylococcus-xylosus-strains-isolated-from-chinese-traditional-low-salt-fermented-whole-fish
#11
Xuefeng Zeng, Laping He, Xu Guo, Li Deng, Wangen Yang, Qiujin Zhu, Zhenhua Duan
This study aimed to determine the predominant processing adaptability of 27 selected isolates of Staphylococcus xylosus in 'Suan yu', a traditional Chinese low-salt fermented whole-fish product. The isolates were screened for proteolytic, lipolytic, and enzymatic profiles; amino-acid decarboxylase content; antimicrobial activities; and tolerance to low temperatures, pH5.0, and salt. Two S. xylosus strains grew at 10°C in the presence of 10% NaCl and at pH5.0. Agar-plate assays and sodium dodecyl sulphate-polyacrylamide gel electrophoresis revealed that 21 and 8 of the strains exhibited appropriate proteolytic activities against myofibrillar and sarcoplasmic proteins, respectively...
February 2, 2017: International Journal of Food Microbiology
https://www.readbyqxmd.com/read/27979096/actin-proteolysis-during-ripening-of-dry-fermented-sausages-at-different-ph-values
#12
A Berardo, B Devreese, H De Maere, D A Stavropoulou, G Van Royen, F Leroy, S De Smet
In dry fermented sausages, myofibrillar proteins undergo intense proteolysis generating small peptides and free amino acids that play a role in flavour generation. This study aimed to identify small peptides arising from actin proteolysis, as influenced by the type of processing. Two acidification profiles were imposed, in order to mimic the pH normally obtained in southern-type and northern-type dry fermented sausages. The identification of peptides was done by liquid chromatography coupled to mass spectrometry in a data-independent positive mode of acquisition (LC-MS(E))...
April 15, 2017: Food Chemistry
https://www.readbyqxmd.com/read/27976420/myopathology-in-congenital-myopathies
#13
Caroline A Sewry, Carina Wallgren-Pettersson
Congenital myopathies are clinically and genetically a heterogeneous group of early onset neuromuscular disorders, characterised by hypotonia and muscle weakness. Clinical severity and age of onset are variable. Many patients are severely affected at birth whilst others have a milder, moderately progressive or non-progressive phenotype. Respiratory weakness is a major clinical aspect that requires regular monitoring. Causative mutations in several genes have been identified that are inherited in a dominant, recessive or X-linked manner or arise de novo...
December 15, 2016: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/27956864/proteome-changes-in-biceps-femoris-muscle-of-iranian%C3%A2-one-humped-camel-and-their-effect-on-meat-quality-traits
#14
Younes Zahedi, Mohammad-Javad Varidi, Mehdi Varidi
In this study physicochemical and quality traits of biceps femoris and longissimus thoracis muscles of male and female Iranian one-humped camel were determined during 14 days of refrigeration storage. Analysis of variance of the results showed that only shear force and temperature were affected by the gender (p<0.05). Anatomical location of the muscle influenced the meat properties except for drip loss (p<0.05). Also, except for cooking loss, ageing influenced the physicochemical and quality properties of meat; during 14 days of storage, proteolysis resulted in an increase of L* and b* values, drip loss and myofibrillar fragmentation index, and the decrease of a* value, expressed juice, shear force and sarcomere length...
September 2016: Food Technology and Biotechnology
https://www.readbyqxmd.com/read/27936702/controlled-cross-linking-with-glucose-oxidase-for-the-enhancement-of-gelling-potential-of-pork-myofibrillar-protein
#15
Xu Wang, Youling L Xiong, Hiroaki Sato, Yoshiyuki Kumazawa
Differential oxidative modifications of myofibrillar protein (MP) by hydroxyl radicals generated in an enzymatic system with glucose oxidase (GluOx) in the presence of glucose/FeSO4 versus a Fenton system (H2O2/FeSO4) were investigated. Pork MP was modified at 4 °C and pH 6.25 with hydroxyl radicals produced from 1 mg/mL glucose in the presence of 80, 160, or 320 μg/mL GluOx and 10 μM FeSO4. Total sulfhydryl content, solubility, cross-linking pattern, and gelation properties of MP were measured. H2O2 production proceeded linearly with the concentration of GluOx and increased with reaction time...
December 21, 2016: Journal of Agricultural and Food Chemistry
https://www.readbyqxmd.com/read/27935521/leucine-supplementation-enhances-integrative-myofibrillar-protein-synthesis-in-free-living-older-men-consuming-lower-and-higher-protein-diets-a-parallel-group-crossover-study
#16
Caoileann H Murphy, Nelson I Saddler, Michaela C Devries, Chris McGlory, Steven K Baker, Stuart M Phillips
BACKGROUND: Leucine co-ingestion with lower-protein (LP)-containing meals may overcome the blunted muscle protein synthetic response to food intake in the elderly but may be effective only in individuals who consume LP diets. OBJECTIVE: We examined the impact of leucine co-ingestion with mixed macronutrient meals on integrated 3-d rates of myofibrillar protein synthesis (MyoPS) in free-living older men who consumed higher protein (HP) (1.2 g · kg(-1) · d(-1)) or LP (0...
December 2016: American Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/27915269/effect-of-ph-on-the-interaction-of-volatile-compounds-with-the-myofibrillar-proteins-of-duck-meat
#17
Q L Yang, X W Lou, Y Wang, D D Pan, Y Y Sun, J X Cao
In order to clarify the influence of curing agents on the flavor of duck, the effect of pH on the surface hydrophobicity, secondary structures, and adsorption capacity of myofibrillar proteins to alcohols, aldehydes, ketones, and esters was assessed using Raman spectroscopy, gas chromatography-mass spectrometer, and other methodologies. The hydrophobicity decreased as pH increased from 5.0 to 8.0; β-turn turned into α-helix and random coil as pH increased from 5.0 to 7.0, while α-helix and random coil turned into β-sheet and β-turn as pH increased from 7...
December 3, 2016: Poultry Science
https://www.readbyqxmd.com/read/27909410/mechanosensitive-molecular-networks-involved-in-transducing-resistance-exercise-signals-into-muscle-protein-accretion
#18
REVIEW
Emil Rindom, Kristian Vissing
Loss of skeletal muscle myofibrillar protein with disease and/or inactivity can severely deteriorate muscle strength and function. Strategies to counteract wasting of muscle myofibrillar protein are therefore desirable and invite for considerations on the potential superiority of specific modes of resistance exercise and/or the adequacy of low load resistance exercise regimens as well as underlying mechanisms. In this regard, delineation of the potentially mechanosensitive molecular mechanisms underlying muscle protein synthesis (MPS), may contribute to an understanding on how differentiated resistance exercise can transduce a mechanical signal into stimulation of muscle accretion...
2016: Frontiers in Physiology
https://www.readbyqxmd.com/read/27908349/truncating-flnc-mutations-are-associated-with-high-risk-dilated-and-arrhythmogenic-cardiomyopathies
#19
Martín F Ortiz-Genga, Sofía Cuenca, Matteo Dal Ferro, Esther Zorio, Ricardo Salgado-Aranda, Vicente Climent, Laura Padrón-Barthe, Iria Duro-Aguado, Juan Jiménez-Jáimez, Víctor M Hidalgo-Olivares, Enrique García-Campo, Chiara Lanzillo, M Paz Suárez-Mier, Hagith Yonath, Sonia Marcos-Alonso, Juan P Ochoa, José L Santomé, Diego García-Giustiniani, Jorge L Rodríguez-Garrido, Fernando Domínguez, Marco Merlo, Julián Palomino, María L Peña, Juan P Trujillo, Alicia Martín-Vila, Davide Stolfo, Pilar Molina, Enrique Lara-Pezzi, Francisco E Calvo-Iglesias, Eyal Nof, Leonardo Calò, Roberto Barriales-Villa, Juan R Gimeno-Blanes, Michael Arad, Pablo García-Pavía, Lorenzo Monserrat
BACKGROUND: Filamin C (encoded by the FLNC gene) is essential for sarcomere attachment to the plasmatic membrane. FLNC mutations have been associated with myofibrillar myopathies, and cardiac involvement has been reported in some carriers. Accordingly, since 2012, the authors have included FLNC in the genetic screening of patients with inherited cardiomyopathies and sudden death. OBJECTIVES: The aim of this study was to demonstrate the association between truncating mutations in FLNC and the development of high-risk dilated and arrhythmogenic cardiomyopathies...
December 6, 2016: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/27906113/dystrophin-restoration-therapy-improves-both-the-reduced-excitability-and-the-force-drop-induced-by-lengthening-contractions-in-dystrophic-mdx-skeletal-muscle
#20
Pauline Roy, Fredérique Rau, Julien Ochala, Julien Messéant, Bodvael Fraysse, Jeanne Lainé, Onnik Agbulut, Gillian Butler-Browne, Denis Furling, Arnaud Ferry
BACKGROUND: The greater susceptibility to contraction-induced skeletal muscle injury (fragility) is an important dystrophic feature and tool for testing preclinic dystrophin-based therapies for Duchenne muscular dystrophy. However, how these therapies reduce the muscle fragility is not clear. METHODS: To address this question, we first determined the event(s) of the excitation-contraction cycle which is/are altered following lengthening (eccentric) contractions in the mdx muscle...
July 20, 2016: Skeletal Muscle
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