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https://www.readbyqxmd.com/read/28104788/myo18b-is-essential-for-sarcomere-assembly-in-fast-skeletal-muscle
#1
Joachim Berger, Silke Berger, Mei Li, Peter D Currie
Congenital myopathies are muscle degenerative disorders with a broad clinical spectrum. A number of myopathies have been associated with molecular defects within sarcomeres, the force-generating component of the muscle cell. Whereas the highly regular organization of the myofibril has been studied in detail, in vivo assembly of sarcomeres remains a poorly understood process. Therefore, a more detailed knowledge of sarcomere assembly is crucial to better understand the pathogenic mechanisms within myopathies...
January 18, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28104714/long-term-biased-%C3%AE-arrestin-signaling-improves-cardiac-structure-and-function-in-dilated-cardiomyopathy
#2
David M Ryba, Jieli Li, Conrad L Cowan, Brenda Russell, Beata M Wolska, R John Solaro
BACKGROUND: -Biased agonism of the angiotensin receptor (AT1R) is known to promote cardiac contractility. Our laboratory indicated that these effects may be due to changes at the level of the myofilaments. However, these signaling mechanisms remain unknown. As a common finding in dilated cardiomyopathy (DCM) is a reduction in the myofilament-Ca(2+)-response, we hypothesized that β-arrestin signaling would increase myofilament-Ca(2+)-responsiveness in a model of familial DCM and improve cardiac function and morphology...
January 19, 2017: Circulation
https://www.readbyqxmd.com/read/28101866/titin-and-nebulin-in-thick-and-thin-filament-length-regulation
#3
Larissa Tskhovrebova, John Trinick
In this review we discuss the history and the current state of ideas related to the mechanism of size regulation of the thick (myosin) and thin (actin) filaments in vertebrate striated muscles. Various hypotheses have been considered during of more than half century of research, recently mostly involving titin and nebulin acting as templates or 'molecular rulers', terminating exact assembly. These two giant, single-polypeptide, filamentous proteins are bound in situ along the thick and thin filaments, respectively, with an almost perfect match in the respective lengths and structural periodicities...
2017: Sub-cellular Biochemistry
https://www.readbyqxmd.com/read/28097712/force-generation-by-titin-folding
#4
Zsolt Mártonfalvi, Pasquale Bianco, Katalin Naftz, György G Ferenczy, Miklós Kellermayer
Titin is a giant protein that provides elasticity to muscle. As the sarcomere is stretched, titin extends hierarchically according to the mechanics of its segments. Whether titin's globular domains unfold during this process and how such unfolded domains might contribute to muscle contractility are strongly debated. To explore the force-dependent folding mechanisms, here we manipulated skeletal-muscle titin molecules with high-resolution optical tweezers. In force-clamp mode, after quenching the force (<10 pN), extension fluctuated without resolvable discrete events...
January 18, 2017: Protein Science: a Publication of the Protein Society
https://www.readbyqxmd.com/read/28096456/antibody-conjugated-dna-based-nanocarriers-intercalated-with-doxorubicin-eliminate-myofibroblasts-in-explants-of-human-lens-tissue
#5
Jacquelyn Gerhart, Marvin Greenbaum, Lou Casta, Anthony Clemente, Keith Mathers, Robert Getts, Mindy George-Weinstein
Posterior capsule opacification (PCO) occurs in some adults and most children following cataract surgery. The fibrotic form of PCO arises, in part, from migratory, contractile myofibroblasts that deform the lens capsule and impair vision. In short-term cultures of human anterior lens tissue, myofibroblasts emerge from Myo/Nog cells that are identified with the G8 monoclonal antibody and by their expression of the MyoD transcription factor and bone morphogenetic protein inhibitor noggin. In this study, we tested the hypothesis that targeted depletion of Myo/Nog cells with the G8 mAb conjugated to three-dimensional DNA nanocarriers intercalated with Doxorubicin (G8:3DNA:Dox) would prevent the accumulation of myofibroblasts in long-term, serum and growth factor free cultures of human lens tissue obtained by capsulorhexis...
January 17, 2017: Journal of Pharmacology and Experimental Therapeutics
https://www.readbyqxmd.com/read/28096043/a-simplified-protocol-for-culture-of-murine-neonatal-cardiomyocytes-on-nanoscale-keratin-coated-surfaces
#6
Aditi Jain, Venkatraman Ravi, Jaseer Muhamed, Kaushik Chatterjee, Nagalingam R Sundaresan
OBJECTIVE: We aim to develop a simple, efficient and cost-effective protocol for culturing the neonatal cardiomyocytes using keratin derived from human hair, which can be used for studying cardiac hypertrophy in vitro. METHODS: Keratin was extracted from human hair and applied as nanoscale coating onto the culture dishes. Physical parameters such as surface morphology and roughness of the coating were studied by SEM and AFM. Cardiomyocyte specific markers were assessed by immunofluorescence...
January 9, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28094777/high-resolution-structural-evidence-suggests-the-sarcoplasmic-reticulum-forms-microdomains-with-acidic-stores-lysosomes-in-the-heart
#7
Daniel Aston, Rebecca A Capel, Kerrie L Ford, Helen C Christian, Gary R Mirams, Eva A Rog-Zielinska, Peter Kohl, Antony Galione, Rebecca A B Burton, Derek A Terrar
Nicotinic Acid Adenine Dinucleotide Phosphate (NAADP) stimulates calcium release from acidic stores such as lysosomes and is a highly potent calcium-mobilising second messenger. NAADP plays an important role in calcium signalling in the heart under basal conditions and following β-adrenergic stress. Nevertheless, the spatial interaction of acidic stores with other parts of the calcium signalling apparatus in cardiac myocytes is unknown. We present evidence that lysosomes are intimately associated with the sarcoplasmic reticulum (SR) in ventricular myocytes; a median separation of 20 nm in 2D electron microscopy and 3...
January 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28091730/actin-isoform-expression-patterns-in-adult-extracardiac-and-cardiac-rhabdomyomas-indicate-a-different-cell-of-origin
#8
Christina C Westhoff, Katharina Schoner, Sylvia Hartmann, Andreas M Sesterhenn, Roland Moll
Rhabdomyomas are rare striated muscle-type tumors arising in the heart or in soft tissues. Using a monoclonal antibody specific for the cardiac isoform of α-actin (α-cardiac actin, α-CAA), differential expression patterns in striated muscle tissues were reported previously. The purpose of the present study was to determine whether the α-actin isoform specificity is maintained in rhabdomyomas according to their origin, comparing extracardiac to cardiac rhabdomyomas. We immunohistochemically investigated adult extracardiac (pharyngeal) rhabdomyomas (n = 4) and cardiac rhabdomyomas (n = 7) employing isoform-specific monoclonal antibodies...
January 14, 2017: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/28090637/diltiazem-prevents-stress-induced-contractile-deficits-in-cardiomyocytes-but-does-not-reverse-the-cardiomyopathy-phenotype-in-mybpc3-knock-in-mice
#9
Frederik Flenner, Birgit Geertz, Silke Reischmann-Düsener, Florian Weinberger, Thomas Eschenhagen, Lucie Carrier, Felix W Friedrich
Left ventricular hypertrophy, diastolic dysfunction and fibrosis are main features of hypertrophic cardiomyopathy (HCM). Guidelines recommend β-adrenoceptor or Ca(2+) channel antagonists as pharmacological treatment. The Ca(2+) channel blocker diltiazem recently showed promising beneficial effects in pre-clinical HCM, particularly in patients carrying MYBPC3 mutations. In the present study we evaluated whether diltiazem could ameliorate or reverse the disease phenotype in cells and in vivo in Mybpc3-targeted knock-in (KI) mouse model of HCM...
January 15, 2017: Journal of Physiology
https://www.readbyqxmd.com/read/28088328/altered-myocyte-contractility-and-calcium-homeostasis-in-alpha-myosin-heavy-chain-point-mutations-linked-to-familial-dilated-cardiomyopathy
#10
Matthew Klos, Lakshmi Mundada, Indroneal Banerjee, Sherry Morgenstern, Stephanie Myers, Michael Leone, Mark Kleid, Todd Herron, Eric Devaney
Mutations in the human cardiac motor protein beta-myosin heavy chain (βMHC) have been long recognized as a cause of familial hypertrophic cardiomyopathy. Recently, mutations (P830L and A1004S) in the less abundant but faster isoform alpha-myosin heavy chain (αMHC) have been linked to dilated cardiomyopathy (DCM). In this study, we sought to determine the cellular contractile phenotype associated with these point mutations. Ventricular myocytes were isolated from 2 month male Sprague Dawley rats. Cells were cultured in M199 media and infected with recombinant adenovirus containing the P830L or the A1004S mutant human αMHC at a MOI of 500 for 18 h...
January 11, 2017: Archives of Biochemistry and Biophysics
https://www.readbyqxmd.com/read/28077621/functional-redundancy-and-non-redundancy-between-two-troponin-c-isoforms-in-drosophila-adult-muscles
#11
Maria B Chechenova, Sara Maes, Sandy T Oas, Cloyce Nelson, Kaveh G Kiani, Anton L Bryantsev, Richard M Cripps
We investigated the functional overlap of two muscle Troponin C (TpnC) genes that are expressed in the adult fruit fly, Drosophila melanogaster: TpnC4 is predominantly expressed in the indirect flight muscles (IFM), whereas TpnC41C is the main isoform in the tergal depressor of the trochanter muscle (TDT or jump muscle). Using CRISPR/Cas9, we created a transgenic line with a homozygous deletion of TpnC41C, and compared its phenotype to a line lacking functional TpnC4 We found that the removal of either of these genes leads to expression of the other isoform in both muscle types...
January 11, 2017: Molecular Biology of the Cell
https://www.readbyqxmd.com/read/28069669/refining-the-molecular-organization-of-the-cardiac-intercalated-disc
#12
REVIEW
Sarah H Vermij, Hugues Abriel, Toon A B van Veen
This review presents an extensively integrated model of the cardiac intercalated disc (ID), a highly orchestrated structure that connects adjacent cardiomyocytes. Classically, three main structures are distinguished: gap junctions (GJs) metabolically and electrically connect cytoplasm of adjacent cardiomyocytes; adherens junctions (AJs) connect the actin cytoskeleton of adjacent cells; and desmosomes function as cell anchors and connect intermediate filaments. Furthermore, ion channels reside in the ID. Mutations in ID proteins have been associated with cardiac arrhythmias such as Brugada syndrome and arrhythmogenic cardiomyopathy...
January 8, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28068629/stars-knockout-attenuates-hypoxia-induced-pulmonary-arterial-hypertension-by-suppressing-pulmonary-arterial-smooth-muscle-cell-proliferation
#13
Zhaoling Shi, Huajie Wu, Jianfeng Luo, Xin Sun
STARS (STriated muscle Activator of Rho Signaling) is a sarcomeric protein, which expressed early in cardiac development and involved in pathological remodeling. Abundant evidence indicated that STARS could regulate cell proliferation, but it's exact function remains unclear. In this study, we aimed to investigate the role of STARS in the proliferation of pulmonary arterial smooth muscle cells (PASMC) and the potential effect on the progression of pulmonary arterial hypertension (PAH). In this study, we established a PAH mouse model through chronic hypoxia exposure as reflected by the increased RVSP and RVHI...
January 6, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28067713/widespread-down-regulation-of-cardiac-mitochondrial-and-sarcomeric-genes-in-patients-with-sepsis
#14
Scot J Matkovich, Belal Al Khiami, Igor R Efimov, Sarah Evans, Justin Vader, Ashwin Jain, Bernard H Brownstein, Richard S Hotchkiss, Douglas L Mann
OBJECTIVES: The mechanism(s) for septic cardiomyopathy in humans is not known. To address this, we measured messenger RNA alterations in hearts from patients who died from systemic sepsis, in comparison to changed messenger RNA expression in nonfailing and failing human hearts. DESIGN: Identification of genes with altered abundance in septic cardiomyopathy, ischemic heart disease, or dilated cardiomyopathy, in comparison to nonfailing hearts. SETTING: ICUs at Barnes-Jewish Hospital, St...
January 6, 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/28065693/inhibition-of-mir-208b-improves-cardiac-function-in-titin-based-dilated-cardiomyopathy
#15
Qifeng Zhou, Sonja Schötterl, Daniel Backes, Eva Brunner, Julia Kelley Hahn, Elena Ionesi, Parwez Aidery, Carsten Sticht, Siegfried Labeit, Reinhard Kandolf, Meinrad Gawaz, Michael Gramlich
BACKGROUND: Dilated cardiomyopathy (DCM) is the result of maladaptive cardiac remodeling, which involves microRNA regulation. In turn, microRNAs can contribute to the remodeling process by post-transcriptional modulation of gene expression networks. The exact role of microRNAs in the pathogenesis of DCM is largely unknown. Here, we used an inducible DCM mouse model that carries a human truncation mutation in the sarcomeric protein titin to dissect microRNA pathways in DCM development...
December 28, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/28065644/in%C3%A2-vivo-maturation-of-human-induced-pluripotent-stem-cell-derived-cardiomyocytes-in-neonatal-and-adult-rat-hearts
#16
Shin Kadota, Lil Pabon, Hans Reinecke, Charles E Murry
We hypothesized that the neonatal rat heart would bring transplanted human induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) to maturity as it grows to adult size. In neonatal rat heart, engrafted hiPSC derivatives developed partially matured myofibrils after 3 months, with increasing cell size and sarcomere length. There was no difference between grafts from hiPSC-CMs or hiPSC-derived cardiac progenitors (hiPSC-CPs) at 3 months, nor was maturation influenced by infarction. Interestingly, the infarcted adult heart induced greater human cardiomyocyte hypertrophy and induction of cardiac troponin I expression than the neonatal heart...
November 22, 2016: Stem Cell Reports
https://www.readbyqxmd.com/read/28062247/severe-apical-hypertrophic-cardiomyopathy-with-ser-236-gly-mutation-in-mybpc3-a-three-year-follow-up-investigation
#17
XueJiang Cen, JianLei Zheng, XueLie Hu, BaiMing Qu
Apical hypertrophic cardiomyopathy (AHCM) is a relatively rare form of hypertrophic cardiomyopathy (HCM) and usually involved with genetic variations encoding sarcomeric proteins. In this report, a 68-year-old male presented with exertional angina and giant negative T-waves in the precordial leads V3-V6 was eventually diagnosed with severe AHCM by echocardiography and left ventriculogram. The entire coding Sequences of the most frequent HCM-causing genes were analyzed. A novel mutation of Ser 236 Gly in myosin-binding protein C (MYBPC3) gene was discovered...
January 3, 2017: Hellenic Journal of Cardiology: HJC, Hellēnikē Kardiologikē Epitheōrēsē
https://www.readbyqxmd.com/read/28050543/morphologic-study-of-different-treatments-for-gastrocnemius-muscle-contusion-in-rats
#18
Ana Carolina Brandt de Macedo, Julye Leiko Ywazaki, Rafael Michel de Macedo, Lucia Noronha, Anna Raquel Silveira Gomes
OBJECTIVE: Evaluate the effects of ultrasound and stretching in morphology after rat muscle contusion. METHODS: Male Wistar rats (n = 35, 8-9 weeks, 271 ± 14 g) were divided into five groups: control group (CG = 3); lesion group (LG = 8); lesion + ultrasound group (LUG = 8); lesion + stretching group (LSG = 8); lesion + ultrasound + stretching group (LUSG = 8). The ultrasound was applied in LUG and LUSG from the third to the seventh day, the dose used was 50% pulsed, 0...
November 2016: Revista Brasileira de Ortopedia
https://www.readbyqxmd.com/read/28049727/allosteric-transmission-along-a-loosely-structured-backbone-allows-a-cardiac-troponin-c-mutant-to-function-with-only-one-ca2-ion
#19
Mayra de A Marques, Jose Renato Pinto, Adolfo H Moraes, Anwar Iqbal, Mariana T Q de Magalhães, Jamila Monteiro, Murilo M Pedrote, Martha M Sorenson, Jerson L Silva, Guilherme A P de Oliveira
Hypertrophic cardiomyopathy (HCM) is one of the most common cardiomyopathies, and a major cause of sudden death in young athletes. The Ca2+ sensor of the sarcomere, cardiac troponin C (cTnC), plays an important role in regulating muscle contraction. Although several cardiomyopathy-causing mutations have been identified in cTnC, limited information about their structural defects has been mapped to the HCM phenotype. Here, we use high-resolution electron-spray ionization mass spectrometry (ESI-MS), Carr-Purcell-Meiboom-Gill relaxation dispersion (CPMG-RD) and affinity measurements of cTnC for the thin filament in reconstituted papillary muscles to provide evidence of an allosteric mechanism in mutant cTnC that may play a role to the HCM phenotype...
January 3, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28040389/homozygous-truncating-mutation-in-prenatally-expressed-skeletal-isoform-of-ttn-gene-results-in-arthrogryposis-multiplex-congenita-and-myopathy-without-cardiac-involvement
#20
Ana Fernández-Marmiesse, M Carmen Carrascosa-Romero, Blanca Alfaro Ponce, Andres Nascimento, Carlos Ortez, Norma Romero, Lourdes Palacios, Cecilia Jimenez-Mallebrera, Cristina Jou, Sofía Gouveia, María L Couce
We report the case of a newborn with arthrogryposis multiplex congenita and severe axial hypotonia without cardiac involvement in which, using a customized targeted next-generation sequencing assay for 64 myopathy-associated genes, we detected a novel homozygous truncating mutation, c.38661_38665del, in exon 197 of the TTN gene that is expressed only in the fetal skeletal isoform. Its pathogenicity is supported by evidence of maternal isodisomy for chromosome 2. Muscle pathology showed fibers with core-like areas devoid of oxidative staining and cytoplasmic bodies...
November 11, 2016: Neuromuscular Disorders: NMD
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