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https://www.readbyqxmd.com/read/27920728/connecting-sarcomere-protein-mutations-to-pathogenesis-in-cardiomyopathies-the-development-of-disease-in-a-dish-models
#1
Rebecca Zaunbrecher, Michael Regnier
Recent technological and protocol developments have greatly increased the ability to utilize stem cells transformed into cardiomyocytes as models to study human heart muscle development and how this is affected by disease associated mutations in a variety of sarcomere proteins. In this perspective we provide an overview of these emerging technologies and how they are being used to create better models of "disease in a dish" for both research and screening assays. We also consider the value of these assays as models to explore the seminal processes in initiation of the disease development and the possibility of early interventions...
2016: Frontiers in Physiology
https://www.readbyqxmd.com/read/27918928/effect-of-a-grazing-period-prior-to-finishing-on-a-high-concentrate-diet-on-meat-quality-from-bulls-and-steers
#2
L Moran, M G O'Sullivan, J P Kerry, B Picard, M McGee, E G O'Riordan, A P Moloney
Bulls and steers (n=60) were assigned to a pre-finishing grazing period and subsequently finished on concentrates or offered concentrates without grazing until slaughter (19months). Colour and pH of longissimus thoracis were measured (48h post-slaughter), and samples collected for proximate composition, collagen, sarcomere length, muscle fibre and enzymatic profile analysis. Steaks for texture, cook loss and sensory were aged (14days). Castration increased intramuscular fat content, cook loss and myosin isoforms IIa and I proportions, and decreased IIx proportion (P<0...
November 29, 2016: Meat Science
https://www.readbyqxmd.com/read/27917819/pulsatile-flow-conditioning-of-three-dimensional-bioengineered-cardiac-ventricle
#3
Nikita M Patel, Ravi K Birla
Current physical stimuli mechanical stretch bioreactor studies focus on conditioning planar and/or tubular engineered cardiac constructs. The current 3D bioreactor models in cardiac tissue engineering use differential pressure loading for structural support as opposed to conditioning. The development of the pulsatile flow conditioned ventricle (PFCV) provides a 3D mechanical stretch conditioning method to generate pump function in the engineered cardiac left ventricle. The study utilizes a chitosan bioengineered open ventricle scaffold, to produce the in vitro PFCV model...
December 5, 2016: Biofabrication
https://www.readbyqxmd.com/read/27914791/aberrant-developmental-titin-splicing-and-dysregulated-sarcomere-length-in-thymosin-%C3%AE-4-knockout-mice
#4
Nicola Smart, Johannes Riegler, Cameron W Turtle, Craig A Lygate, Debra J McAndrew, Katja Gehmlich, Karina N Dubé, Anthony N Price, Vivek Muthurangu, Andrew M Taylor, Mark F Lythgoe, Charles Redwood, Paul R Riley
Sarcomere assembly is a highly orchestrated and dynamic process which adapts, during perinatal development, to accommodate growth of the heart. Sarcomeric components, including titin, undergo an isoform transition to adjust ventricular filling. Many sarcomeric genes have been implicated in congenital cardiomyopathies, such that understanding developmental sarcomere transitions will inform the aetiology and treatment. We sought to determine whether Thymosin β4 (Tβ4), a peptide that regulates the availability of actin monomers for polymerization in non-muscle cells, plays a role in sarcomere assembly during cardiac morphogenesis and influences adult cardiac function...
November 30, 2016: Journal of Molecular and Cellular Cardiology
https://www.readbyqxmd.com/read/27913119/mechanical-forces-during-muscle-development
#5
REVIEW
Sandra B Lemke, Frank Schnorrer
Muscles are the major force producing tissue in the human body. While certain muscle types specialise in producing maximum forces, others are very enduring. An extreme example is the heart, which continuously beats for the entire life. Despite being specialised, all body muscles share similar contractile mini-machines called sarcomeres that are organised into regular higher order structures called myofibrils. The major sarcomeric components and their organisational principles are conserved throughout most of the animal kingdom...
November 29, 2016: Mechanisms of Development
https://www.readbyqxmd.com/read/27909224/sarcomeric-protein-modification-during-adrenergic-stress-enhances-cross-bridge-kinetics-and-cardiac-output
#6
Kenneth S Gresham, Ranganath Mamidi, Jiayang Li, Hyerin Kwak, Julian E Stelzer
Molecular adaptations to chronic neurohormonal stress, including sarcomeric protein cleavage and phosphorylation, provide a mechanism to increase ventricular contractility and enhance cardiac output, yet the link between sarcomeric protein modifications and changes in myocardial function remains unclear. To examine the effects of neurohormonal stress on post-translational modifications of sarcomeric proteins, mice were administered combined α- and β-adrenergic receptor agonists (isoproterenol and phenylephrine; IPE) for 14 days using implantable osmotic pumps...
December 1, 2016: Journal of Applied Physiology
https://www.readbyqxmd.com/read/27908349/truncating-flnc-mutations-are-associated-with-high-risk-dilated-and-arrhythmogenic-cardiomyopathies
#7
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/27906069/the-beneficial-role-of-proteolysis-in-skeletal-muscle-growth-and-stress-adaptation
#8
REVIEW
Ryan A V Bell, Mohammad Al-Khalaf, Lynn A Megeney
Muscle atrophy derived from excessive proteolysis is a hallmark of numerous disease conditions. Accordingly, the negative consequences of skeletal muscle protein breakdown often overshadow the critical nature of proteolytic systems in maintaining normal cellular function. Here, we discuss the major cellular proteolysis machinery-the ubiquitin/proteosome system, the autophagy/lysosomal system, and caspase-mediated protein cleavage-and the critical role of these protein machines in establishing and preserving muscle health...
April 6, 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
#9
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/27898874/technical-note-the-effect-of-carcass-deboning-technique-on-the-meat-quality-of-cattle
#10
B Cui, Y Zhang, F Wang, M A Jamali, L Wei, Z Peng
The objective of this study was to evaluate the effects of the suspended carcass deboning technique on the meat attributes of electrically stimulated cattle ( = 10). A carcass deboning technique that removes the main bones without breakdown of the entire carcass was applied to suspended sides soon after slaughter. After 3 d of aging at 4°C, the shear force of the rectus femoris, longissimus lumborum, and supraspinatus muscles of the suspended carcass deboning technique was reduced by 27%, 29%, and 23% ( < 0...
October 2016: Journal of Animal Science
https://www.readbyqxmd.com/read/27890729/maturation-of-human-embryonic-stem-cell-derived-cardiomyocytes-hesc-cms-in-3d-collagen-matrix-effects-of-niche-cell-supplementation-and-mechanical-stimulation
#11
W Zhang, C W Kong, M H Tong, W H Chooi, N Huang, R A Li, B P Chan
: Cardiomyocytes derived from human embryonic stem cells (hESC-CMs) are regarded as a promising source for regenerative medicine, drug testing and disease modeling. Nevertheless, cardiomyocytes are immature in terms of their contractile structure, metabolism and electrophysiological properties. Here, we fabricate cardiac muscle strips by encapsulating hESC-CMs in collagen-based biomaterials. Supplementation of niche cells at 3% to the number of hESC-CMs enhance the maturation of the hESC-CMs in 3D tissue matrix...
November 24, 2016: Acta Biomaterialia
https://www.readbyqxmd.com/read/27889803/reducing-rbm20-activity-improves-diastolic-dysfunction-and-cardiac-atrophy
#12
Florian Hinze, Christoph Dieterich, Michael H Radke, Henk Granzier, Michael Gotthardt
: Impaired diastolic filling is a main contributor to heart failure with preserved ejection fraction (HFpEF), a syndrome with increasing prevalence and no treatment. Both collagen and the giant sarcomeric protein titin determine diastolic function. Since titin's elastic properties can be adjusted physiologically, we evaluated titin-based stiffness as a therapeutic target. We adjusted RBM20-dependent cardiac isoform expression in the titin N2B knockout mouse with increased ventricular stiffness...
November 26, 2016: Journal of Molecular Medicine: Official Organ of the "Gesellschaft Deutscher Naturforscher und Ärzte"
https://www.readbyqxmd.com/read/27888773/the-combined-effects-of-grain-supplementation-and-tenderstretching-on-alpaca-vicugna-pacos-meat-quality
#13
M A Smith, R D Bush, R J van de Ven, D L Hopkins
This study investigated the effects of feeding a mixed grain supplement and tenderstretching (TS) alpaca carcasses on meat quality. A total of 56 castrated 24month old alpacas were divided into two treatments (pasture-only, and pasture plus supplementation). Supplemented groups were fed a mixed grain ration in addition to ad lib pasture for 10weeks. Animals were slaughtered across two kill days (n=28). One half of each carcass was suspended by the pelvis (TS) prior to chilling, and the other half was Achilles tendon hung (AH)...
November 21, 2016: Meat Science
https://www.readbyqxmd.com/read/27885498/prognostic-predictive-value-of-gene-mutations-in-japanese-patients-with-hypertrophic-cardiomyopathy
#14
Ayako Chida, Kei Inai, Hiroki Sato, Eriko Shimada, Tsutomu Nishizawa, Mitsuyo Shimada, Michiko Furutani, Yoshiyuki Furutani, Yoichi Kawamura, Masaya Sugimoto, Jun Ishihara, Masako Fujiwara, Takashi Soga, Masatoshi Kawana, Shinya Fuji, Shigeru Tateno, Kenji Kuraishi, Shigetoyo Kogaki, Mitsuhiro Nishimura, Mamoru Ayusawa, Fukiko Ichida, Hirokuni Yamazawa, Rumiko Matsuoka, Shigeaki Nonoyama, Toshio Nakanishi
Although some studies have attempted to find useful prognostic factors in hypertrophic cardiomyopathy (HCM), those results are not fully helpful for use in actual clinical practice. Furthermore, several genetic abnormalities associated with HCM have been identified. However, the genotype-phenotype correlation in HCM remains to be elucidated. Here, we attempted to assess patients with different types of gene mutations causing HCM and investigate the prognosis. A total of 140 patients with HCM underwent a screening test for myofilament gene mutations by direct sequencing of eight sarcomeric genes...
November 24, 2016: Heart and Vessels
https://www.readbyqxmd.com/read/27882462/proliferative-potential-of-cardiomyocytes-in-hypertrophic-cardiomyopathy-correlation-with-myocardial-remodeling
#15
T V Sukhacheva, Yu A Chudinovskikh, M V Eremeeva, R A Serov, L A Bockeria
Proliferating Ki-67(+) cardiomyocytes were detected in the interventricular septum myocardium of adult patients with hypertrophic cardiomyopathy. In the same patients, the severity of hypertrophy and the degree of cardiomyocyte differentiation were assessed by the content of myofibrils, ultrastructural morphology, and the pattern of connexin 43-containing gap junction distribution. Adult Ki-67(+) cardiomyocytes containing sarcomeric α-actin (sarc α-act(+)) in the sarcoplasm (diameter 23.9±6.9 μ) were detected in the myocardium of patients with hypertrophic cardiomyopathy; their relative content varied from 2 to 3084 cells per 1 million cardiomyocytes...
November 23, 2016: Bulletin of Experimental Biology and Medicine
https://www.readbyqxmd.com/read/27881552/cardiac-t-tubule-microanatomy-and-function
#16
REVIEW
TingTing Hong, Robin M Shaw
Unique to striated muscle cells, transverse tubules (t-tubules) are membrane organelles that consist of sarcolemma penetrating into the myocyte interior, forming a highly branched and interconnected network. Mature t-tubule networks are found in mammalian ventricular cardiomyocytes, with the transverse components of t-tubules occurring near sarcomeric z-discs. Cardiac t-tubules contain membrane microdomains enriched with ion channels and signaling molecules. The microdomains serve as key signaling hubs in regulation of cardiomyocyte function...
January 2017: Physiological Reviews
https://www.readbyqxmd.com/read/27879346/the-zebrafish-frozen-mutant-is-a-model-for-human-myopathy-associated-with-mutation-of-the-unconventional-myosin-myo18b
#17
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/27875570/towards-a-tissue-engineered-contractile-fontan-conduit-the-fate-of-cardiac-myocytes-in-the-subpulmonary-circulation
#18
Daniel Biermann, Alexandra Eder, Florian Arndt, Hatim Seoudy, Hermann Reichenspurner, Thomas Mir, Arlindo Riso, Rainer Kozlik-Feldmann, Kersten Peldschus, Michael G Kaul, Tillman Schuler, Susanne Krasemann, Arne Hansen, Thomas Eschenhagen, Jörg S Sachweh
The long-term outcome of patients with single ventricles improved over time, but remains poor compared to other congenital heart lesions with biventricular circulation. Main cause for this unfavourable outcome is the unphysiological hemodynamic of the Fontan circulation, such as subnormal systemic cardiac output and increased systemic-venous pressure. To overcome this limitation, we are developing the concept of a contractile extracardiac Fontan-tunnel. In this study, we evaluated the survival and structural development of a tissue-engineered conduit under in vivo conditions...
2016: PloS One
https://www.readbyqxmd.com/read/27872154/genetic-determinants-of-myocardial-dysfunction
#19
REVIEW
Xianchi Li, Peiying Zhang
Heart failure (HF) is a major killer with high morbidity and mortality and nearly 37.7 million people are affected by HF globally, making this a global epidemic. HF is a complex pathophysiological syndrome in which the mechanical function of heart for pumping blood is compromised. Cardiac structural and functional abnormalities culminate in decreased cardiac output along with increased intracardiac pressures under resting or stress conditions, leading to HF. Besides the acquired risk factors, the independent role of hereditary and genetic factors in the development, progression and prognosis of HF remains to be established...
November 21, 2016: Journal of Medical Genetics
https://www.readbyqxmd.com/read/27870901/ubiquitin-dependent-modification-of-skeletal-muscle-by-the-parasitic-nematode-trichinella-spiralis
#20
Rhiannon R White, Amy H Ponsford, Michael P Weekes, Rachel B Rodrigues, David B Ascher, Marco Mol, Murray E Selkirk, Steven P Gygi, Christopher M Sanderson, Katerina Artavanis-Tsakonas
Trichinella spiralis is a muscle-specific parasitic worm that is uniquely intracellular. T. spiralis reprograms terminally differentiated skeletal muscle cells causing them to de-differentiate and re-enter the cell cycle, a process that cannot occur naturally in mammalian skeletal muscle cells, but one that holds great therapeutic potential. Although the host ubiquitin pathway is a common target for viruses and bacteria during infection, its role in parasite pathogenesis has been largely overlooked. Here we demonstrate that the secreted proteins of T...
November 2016: PLoS Pathogens
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