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Myofibrillar

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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
#1
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
#2
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
#3
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
#4
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
#5
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
https://www.readbyqxmd.com/read/27904835/a-novel-dominant-d109a-cryab-mutation-in-a-family-with-myofibrillar-myopathy-affects-%C3%AE-b-crystallin-structure
#6
Jakub P Fichna, Anna Potulska-Chromik, Przemysław Miszta, Maria Jolanta Redowicz, Anna M Kaminska, Cezary Zekanowski, Sławomir Filipek
Myofibrillar myopathy (MFM) is a group of inherited muscular disorders characterized by myofibrils dissolution and abnormal accumulation of degradation products. So far causative mutations have been identified in nine genes encoding Z-disk proteins, including αB-crystallin (CRYAB), a small heat shock protein (also called HSPB5). Here, we report a case study of a 63-year-old Polish female with a progressive lower limb weakness and muscle biopsy suggesting a myofibrillar myopathy, and extra-muscular multisystemic involvement, including cataract and cardiomiopathy...
June 2017: BBA Clinical
https://www.readbyqxmd.com/read/27897407/activin-a-induces-skeletal-muscle-catabolism-via-p38%C3%AE-mitogen-activated-protein-kinase
#7
Hui Ding, Guohua Zhang, Ka Wai Thomas Sin, Zhelong Liu, Ren-Kuo Lin, Min Li, Yi-Ping Li
BACKGROUND: Activation of type IIB activin receptor (ActRIIB) in skeletal muscle leads to muscle atrophy because of increased muscle protein degradation. However, the intracellular signalling mechanism that mediates ActRIIB-activated muscle catabolism is poorly defined. METHODS: We investigated the role of p38β mitogen-activated protein kinases (MAPK) in mediating ActRIIB ligand activin A-activated muscle catabolic pathways in C2C12 myotubes and in mice with perturbation of this kinase pharmacologically and genetically...
September 16, 2016: Journal of Cachexia, Sarcopenia and Muscle
https://www.readbyqxmd.com/read/27879346/the-zebrafish-frozen-mutant-is-a-model-for-human-myopathy-associated-with-mutation-of-the-unconventional-myosin-myo18b
#8
Ritika Gurung, Yosuke Ono, Sarah Baxendale, Samantha Lin Chiou Lee, Steven Moore, Meredith Calvert, Philip W Ingham
MYOSIN 18B is an unconventional myosin that has been implicated in tumour progression in humans. In addition, loss-of-function mutations of the MYO18B gene have recently been identified in several patients exhibiting symptoms of nemaline myopathy. In mouse, mutation of Myo18B results in early developmental arrest associated with cardiomyopathy, precluding analysis of its effects on skeletal muscle development. The zebrafish, frozen (fro) mutant was identified as one of a group of immotile mutants in the 1996 Tübingen genetic screen...
November 22, 2016: Genetics
https://www.readbyqxmd.com/read/27875560/denervation-induced-activation-of-the-standard-proteasome-and-immunoproteasome
#9
Haiming M Liu, Deborah A Ferrington, Cory W Baumann, LaDora V Thompson
The standard 26S proteasome is responsible for the majority of myofibrillar protein degradation leading to muscle atrophy. The immunoproteasome is an inducible form of the proteasome. While its function has been linked to conditions of atrophy, its contribution to muscle proteolysis remains unclear. Therefore, the purpose of this study was to determine if the immunoproteasome plays a role in skeletal muscle atrophy induced by denervation. Adult male C57BL/6 wild type (WT) and immunoproteasome knockout lmp7-/-/mecl-1-/- (L7M1) mice underwent tibial nerve transection on the left hindlimb for either 7 or 14 days, while control mice did not undergo surgery...
2016: PloS One
https://www.readbyqxmd.com/read/27870699/resistance-training-alters-the-proportion-of-skeletal-muscle-fibers-but-not-brain-neurotrophic-factors-in-young-adult-rats
#10
José Antonio-Santos, Diórginis José S Ferreira, Gizelle L Gomes Costa, Rhowena Jane B Matos, Ana E Toscano, Raul Manhães-de-Castro, Carol G Leandro
Antonio-Santos, J, Ferreira, DJS, Gomes Costa, GL, Matos, RJB, Toscano, AE, Manhães-de-Castro, R, and Leandro, CG. Resistance training alters the proportion of skeletal muscle fibers but not brain neurotrophic factors in young adult rats. J Strength Cond Res 30(12): 3531-3538, 2016-Resistance training (RT) is related to improved muscular strength and power output. Different programs of RT for rats have been developed, but peripheral and central response has not been evaluated directly in the same animal. To test the hypothesis that RT induces central and peripheral adaptations, this study evaluated the effects of a RT on the performance of a weekly maximum overload test, fiber-type typology, and brain neurotrophic factors in young adult rats...
December 2016: Journal of Strength and Conditioning Research
https://www.readbyqxmd.com/read/27869319/the-ca-2-sensitizer-ck-2066260-increases-myofibrillar-ca-2-sensitivity-and-submaximal-force-selectively-in-fast-skeletal-muscle
#11
Darren T Hwee, Arthur J Cheng, James J Hartman, Aaron C Hinken, Ken Lee, Nickie Durham, Alan J Russell, Fady I Malik, Håkan Westerblad, Jeffrey R Jasper
Skeletal muscle dysfunction occurs in many diseases and can lead to muscle weakness and premature muscle fatigue. Here we show that the fast skeletal troponin activator, CK-2066260, counteracts muscle weakness by increasing troponin Ca(2+) affinity, thereby increasing myofibrillar Ca(2+) sensitivity. Exposure to CK-2066260 resulted in a concentration-dependent increase in the Ca(2+) sensitivity of ATPase activity in isolated myofibrils and reconstituted hybrid sarcomeres containing fast skeletal muscle troponin C...
November 21, 2016: Journal of Physiology
https://www.readbyqxmd.com/read/27857544/effects-of-red-bean-vigna-angularis-protein-isolates-on-rheological-properties-of-microbial-transglutaminase-mediated-pork-myofibrillar-protein-gels-as-affected-by-fractioning-and-preheat-treatment
#12
Ho Sik Jang, Hong Chul Lee, Koo Bok Chin
Fractioning and/or preheating treatment on the rheological properties of myofibrillar protein (MP) gels induced by microbial transglutaminase (MTG) has been reported that they may improve the functional properties. However, the optimum condition was varied depending on the experimental factors. This study was to evaluate the effect of red bean protein isolate (RBPI) on the rheological properties of MP gels mediated by MTG as affected by modifications (fractioning: 7S-globulin of RBPI and/or preheat treatment (pre-heating; 95℃/30 min): pre-heating RBPI or pre-heating/7S-globulin)...
October 31, 2016: Korean Journal for Food Science of Animal Resources
https://www.readbyqxmd.com/read/27855606/european-carp-cyprinus-carpio-l-protein-derived-ex-vivo-digests-and-in-vitro-hydrolysates-differ-in-the-ace-i-inhibitory-activity-and-composition-of-released-ace-inhibitory-peptides
#13
Małgorzata Darewicz, Justyna Borawska-Dziadkiewicz, Gerd E Vegarud, Piotr Minkiewicz
Carp muscle tissue is a valuable source of biologically active constituents known to positively influence human health. In this study, carp protein digests/hydrolysates generated by human/porcine digestive enzymes were analysed for their angiotensin I-converting enzyme inhibitory (ACEi) activity. The ex vivo digests and in vitro hydrolysates were used in a screening for ACEi peptides as well. Carp proteins were hydrolysed more rapidly by human gastrointestinal juices than by porcine enzymes. Sarcoplasmic protein fractions were digested/hydrolysed more facile than the myofibrillar ones...
November 17, 2016: Protein and Peptide Letters
https://www.readbyqxmd.com/read/27854226/neo-epitope-peptides-as-biomarkers-of-disease-progression-for-muscular-dystrophies-and-other-myopathies
#14
A Arvanitidis, K Henriksen, M A Karsdal, A Nedergaard
For several decades, serological biomarkers of neuromuscular diseases as dystrophies, myopathies and myositis have been limited to routine clinical biochemistry panels. Gauging the pathological progression is a prerequisite for proper treatment and therefore identifying accessible, easy to monitor biomarkers that can predict the disease progression would be an important advancement. Most muscle diseases involve accelerated muscle fiber degradation, inflammation, fatty tissue substitution and/or fibrosis. All these pathological traits have been shown to give rise to serological peptide biomarkers in other tissues, underlining the potential application of existing biomarkers of such traits in muscle disorders...
August 30, 2016: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/27854214/homozygosity-of-the-dominant-myotilin-c-179c-t-p-ser60phe-mutation-causes-a-more-severe-and-proximal-muscular-dystrophy
#15
Gabrielle Rudolf, Tiina Suominen, Sini Penttilä, Peter Hackman, Anni Evilä, Béatrice Lannes, Andoni Echaniz-Laguna, Guillaume Bierry, Christine Tranchant, Bjarne Udd
Most myotilinopathy patients present with a dominant late onset distal phenotype and myofibrillar pathology, although the first MYOT mutation in a family reported to have LGMD phenotype. We report here a French family affected with a late onset proximal and distal muscle weakness and myofibrillar myopathy on muscle pathology, in which the siblings known to be clinically affected were homozygous for the c.179C>T (p.Ser60Phe) myotilin gene mutation. One subjectively asymptomatic member of the family was heterozygous for this mutation...
May 27, 2016: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/27833457/itraq-based-proteomic-analysis-of-myofibrillar-contents-and-relevant-synthesis-and-proteolytic-proteins-in-soleus-muscle-of-hibernating-daurian-ground-squirrels-spermophilus-dauricus
#16
Hui Chang, Shan-Feng Jiang, Kai Dang, Hui-Ping Wang, Shen-Hui Xu, Yun-Fang Gao
BACKGROUND: Daurian ground squirrels (Spermophilus dauricus) deviate from significant increase of protein catabolism and loss of myofibrillar contents during long period of hibernation inactivity. METHODS: Here we use iTRAQ based quantitative analysis to examine proteomic changes in the soleus of squirrels in pre-hibernation, hibernation and post-hibernation states. The total proteolysis rate of soleus was measured by the release of the essential amino acid tyrosine from isolated muscles...
2016: Proteome Science
https://www.readbyqxmd.com/read/27832381/assembly-and-maintenance-of-myofibrils-in-striated-muscle
#17
Joseph W Sanger, Jushuo Wang, Yingli Fan, Jennifer White, Lei Mi-Mi, Dipak K Dube, Jean M Sanger, David Pruyne
In this chapter, we present the current knowledge on de novo assembly, growth, and dynamics of striated myofibrils, the functional architectural elements developed in skeletal and cardiac muscle. The data were obtained in studies of myofibrils formed in cultures of mouse skeletal and quail myotubes, in the somites of living zebrafish embryos, and in mouse neonatal and quail embryonic cardiac cells. The comparative view obtained revealed that the assembly of striated myofibrils is a three-step process progressing from premyofibrils to nascent myofibrils to mature myofibrils...
November 11, 2016: Handbook of Experimental Pharmacology
https://www.readbyqxmd.com/read/27816332/widening-the-spectrum-of-filamin-c-myopathy-predominantly-proximal-myopathy-due-to-the-p-a193t-mutation-in-the-actin-binding-domain-of-flnc
#18
Fleur J A van den Bogaart, Kristl G Claeys, Rudolf A Kley, Benno Kusters, Simone Schrading, Erik J Kamsteeg, Nicol C Voermans
We report three patients with a predominantly proximal myopathy due to p.A193T mutation in the actin-binding domain of FLNC, which has so far only been associated with a distal myopathy. They presented with a late onset myopathy characterized by predominant limb-girdle and proximal weakness. We describe the clinical, electrophysiological, pathological, muscle imaging and genetic features. One of our patients did not have typical histological features for a myofibrillar myopathy in muscle biopsy. This observation is important for the recognition of the full clinical spectrum of filamin-C-related myopathies...
September 27, 2016: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/27790792/als-associated-endoplasmic-reticulum-proteins-in-denervated-skeletal-muscle-implications-for-motor-neuron-disease-pathology
#19
C M Jesse, E Bushuven, P Tripathi, A Chandrasekar, C M Simon, C Drepper, A Yamoah, A Dreser, I Katona, S Johann, C Beyer, S Wagner, M Grond, S Nikolin, J Anink, D Troost, M Sendtner, A Goswami, J Weis
Alpha-motoneurons and muscle fibers are structurally and functionally interdependent. Both cell types particularly rely on endoplasmic reticulum (ER/SR) functions. Mutations of the ER proteins VAPB, SigR1 and HSP27 lead to hereditary motor neuron diseases (MNDs). Here, we determined the expression profile and localization of these ER proteins/chaperons by immunohistochemistry and immunoblotting in biopsy and autopsy muscle tissue of patients with amyotrophic lateral sclerosis (ALS) and other neurogenic muscular atrophies (NMAs) and compared these patterns to mouse models of neurogenic muscular atrophy...
October 28, 2016: Brain Pathology
https://www.readbyqxmd.com/read/27790050/arrhythmogenic-right-ventricular-dysplasia-in-neuromuscular-disorders
#20
REVIEW
Josef Finsterer, Claudia Stöllberger
OBJECTIVES: Arrhythmogenic right ventricular dysplasia (ARVD) is a rare, genetic disorder predominantly affecting the right ventricle. There is increasing evidence that in some cases, ARVD is due to mutations in genes, which have also been implicated in primary myopathies. This review gives an overview about myopathy-associated ARVD and how these patients can be managed. METHODS: A literature review was done using appropriate search terms. RESULTS: The myopathy, which is most frequently associated with ARVD, is the myofibrillar myopathy due to desmin mutations...
2016: Clinical Medicine Insights. Cardiology
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