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https://www.readbyqxmd.com/read/28915917/translocation-of-molecular-chaperones-to-the-titin-springs-is-common-in-skeletal-myopathy-patients-and-affects-sarcomere-function
#1
Andreas Unger, Lisa Beckendorf, Pierre Böhme, Rudolf Kley, Marion von Frieling-Salewsky, Hanns Lochmüller, Rolf Schröder, Dieter O Fürst, Matthias Vorgerd, Wolfgang A Linke
Myopathies encompass a wide variety of acquired and hereditary disorders. The pathomechanisms include structural and functional changes affecting, e.g., myofiber metabolism and contractile properties. In this study, we observed increased passive tension (PT) of skinned myofibers from patients with myofibrillar myopathy (MFM) caused by FLNC mutations (MFM-filaminopathy) and limb-girdle muscular dystrophy type-2A due to CAPN3 mutations (LGMD2A), compared to healthy control myofibers. Because the giant protein titin determines myofiber PT, we measured its molecular size and the titin-to-myosin ratio, but found no differences between myopathies and controls...
September 15, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/28887874/small-molecule-inhibition-of-murf1-attenuates-skeletal-muscle-atrophy-and-dysfunction-in-cardiac-cachexia
#2
Thomas Scott Bowen, Volker Adams, Sarah Werner, Tina Fischer, Paulien Vinke, Maria Noel Brogger, Norman Mangner, Axel Linke, Peter Sehr, Joe Lewis, Dittmar Labeit, Alexander Gasch, Siegfried Labeit
BACKGROUND: Muscle ring finger 1 (MuRF1) is a muscle-specific ubiquitin E3 ligase activated during clinical conditions associated with skeletal muscle wasting. Yet, there remains a paucity of therapeutic interventions that directly inhibit MuRF1 function, particularly in vivo. The current study, therefore, developed a novel compound targeting the central coiled coil domain of MuRF1 to inhibit muscle wasting in cardiac cachexia. METHODS: We identified small molecules that interfere with the MuRF1-titin interaction from a 130 000 compound screen based on Alpha Technology...
September 8, 2017: Journal of Cachexia, Sarcopenia and Muscle
https://www.readbyqxmd.com/read/28866075/between-disease-causing-and-an-innocent-bystander-the-role-of-titin-as-a-modifier-in-hypertrophic-cardiomyopathy
#3
EDITORIAL
Brenda Gerull
No abstract text is available yet for this article.
July 21, 2017: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/28860074/hepatopancreas-transcriptome-analysis-of-chinese-mitten-crab-eriocheir-sinensis-with-white-hepatopancreas-syndrome
#4
Xiaowen Chen, Jun Wang, Wucheng Yue, Jinsheng Liu, Chenghui Wang
White hepatopancreas is a syndrome that has recently emerged in aquaculture of Chinese mitten crab (Eriocheir sinensis). High lethality of the disease caused large economic loss, which drew considerable attention of fish farmers and scientific researchers. In this study, hepatopancreas reference transcriptome was de novo assembled and differential expression analysis was conducted between white hepatopancreas and normal (yellow) hepatopancreas of E. sinensis. A total of 90,687 transcripts were assembled, and 27,387 were annotated...
August 30, 2017: Fish & Shellfish Immunology
https://www.readbyqxmd.com/read/28854511/neonatal-complex-arrhythmias-possibly-related-to-a-ttn-mutation
#5
William N Evans, Ruben J Acherman, Ian H Law, Nicholas H Von Bergen, Ricardo A Samson, Humberto Restrepo
We describe a neonate born with complex arrhythmias that included concurrent atrial and ventricular tachycardias. Genetic testing demonstrated a mutation in the TTN gene, which codes for titin, a large protein found in striated muscle sarcomeres. The complex arrhythmias were successfully treated with amiodarone and flecainide. The patient remains asymptomatic with normal biventricular function. We speculate that the complex arrhythmias and TTN gene mutation may be related.
August 23, 2017: Journal of Neonatal-perinatal Medicine
https://www.readbyqxmd.com/read/28826663/exercise-responsive-phosphoproteins-in-the-heart
#6
Hongbo Guo, Ruth Isserlin, Andrew Emili, Jatin G Burniston
Endurance exercise improves cardiac performance and affords protection against cardiovascular diseases but the signalling events that mediate these benefits are largely unexplored. Phosphorylation is a widely studied post-translational modification involved in intracellular signalling, and to discover novel phosphorylation events associated with exercise we have profiled the cardiac phosphoproteome response to a standardised exercise test to peak oxygen uptake (VO2peak). Male Wistar rats (346±18g) were assigned to 3 independent groups (n=6, in each) that were familiarised with running on a motorised treadmill within a metabolic chamber...
August 4, 2017: Journal of Molecular and Cellular Cardiology
https://www.readbyqxmd.com/read/28822653/titin-truncating-variants-increase-the-risk-of-cardiovascular-death-in-patients-with-hypertrophic-cardiomyopathy
#7
Ce Zhang, Hongju Zhang, Guixin Wu, Xiaoliang Luo, Channa Zhang, Yubao Zou, Hu Wang, Rutai Hui, Jizheng Wang, Lei Song
BACKGROUND: Titin-truncating variants (TTNtv) have been detected in a variety of cardiomyopathies and represent the most common cause of dilated cardiomyopathy. However, their significance in hypertrophic cardiomyopathy (HCM) is still unclear. METHODS: The titin gene (TTN) was sequenced for truncating variants in a cohort of 529 Chinese patients with HCM and 307 healthy controls. Baseline and follow-up clinical data (for 4.7 ± 3.2 years) from these patients were obtained...
June 8, 2017: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/28811266/expression-and-purification-of-a-difficult-sarcomeric-protein-telethonin
#8
Huanbo Tan, Wencheng Su, Pengju Wang, Wenyu Zhang, Michael Sattler, Peijian Zou
Telethonin anchors the N-terminal region of titin in the Z-disk of the sarcomere by binding to two immunoglobulin-like (Ig) domains (Z1 and Z2) of titin (Z1Z2). Thereby telethonin plays an important role in myofibril assembly and in muscle development and functional regulation. The expression and purification of recombinant telethonin is very challenging. In previous studies, recombinant telethonin expressed from E. coli was refolded in the presence of Z1Z2. Here, we report various strategies to establish a reliable and efficient protocol for the preparation of telethonin and titin Z1Z2 protein...
August 12, 2017: Protein Expression and Purification
https://www.readbyqxmd.com/read/28801508/intricate-and-cell-type-specific-populations-of-endogenous-circular-dna-eccdna-in-caenorhabditis-elegans-and-homo-sapiens
#9
Massa J Shoura, Idan Gabdank, Loren Hansen, Jason Merker, Jason Gotlib, Stephen D Levene, Andrew Z Fire
Investigations aimed at defining the 3-D configuration of eukaryotic chromosomes have consistently encountered an endogenous population of chromosome-derived circular genomic DNA, referred to as extrachromosomal circular DNA (eccDNA). While the production, distribution, and activities of eccDNAs remain understudied, eccDNA formation from specific regions of the linear genome has profound consequences on the regulatory and coding capabilities for these regions. Here, we define eccDNA distributions in C. elegans and in three human cell types, utilizing a set of DNA-topology dependent approaches for enrichment and characterization...
August 11, 2017: G3: Genes—Genomes—Genetics
https://www.readbyqxmd.com/read/28800592/cx3cr1-knockout-aggravates-coxsackievirus-b3-induced-myocarditis
#10
Irene Müller, Kathleen Pappritz, Konstantinos Savvatis, Kerstin Puhl, Fengquan Dong, Muhammad El-Shafeey, Nazha Hamdani, Isabell Hamann, Michel Noutsias, Carmen Infante-Duarte, Wolfgang A Linke, Sophie Van Linthout, Carsten Tschöpe
Studies on inflammatory disorders elucidated the pivotal role of the CX3CL1/CX3CR1 axis with respect to the pathophysiology and diseases progression. Coxsackievirus B3 (CVB3)-induced myocarditis is associated with severe cardiac inflammation, which may progress to heart failure. We therefore investigated the influence of CX3CR1 ablation in the model of acute myocarditis, which was induced by inoculation with 5x105 plaque forming units of CVB3 (Nancy strain) in either CX3CR1-/- or C57BL6/j (WT) mice. Seven days after infection, myocardial inflammation, remodeling, and titin expression and phosphorylation were examined by immunohistochemistry, real-time PCR and Pro-Q diamond stain...
2017: PloS One
https://www.readbyqxmd.com/read/28800153/increased-autolysis-of-%C3%AE-calpain-in-skeletal-muscles-of-chronic-alcohol-fed-rats
#11
Yulia V Gritsyna, Nikolay N Salmov, Alexander G Bobylev, Anna D Ulanova, Nikolay I Kukushkin, Zoya A Podlubnaya, Ivan M Vikhlyantsev
BACKGROUND: Proteolysis can proceed via several distinct pathways such as the lysosomal, calcium-dependent, and ubiquitin (Ub)-proteasome-dependent pathways. Calpains are the main proteases that cleave a large variety of proteins, including the giant sarcomeric proteins titin and nebulin. Chronic ethanol feeding for 6 weeks did not affect the activities of μ-calpain and m-calpain in the m. gastrocnemius. In our research, changes in μ-calpain activity were studied in the m. gastrocnemius and m...
August 11, 2017: Alcoholism, Clinical and Experimental Research
https://www.readbyqxmd.com/read/28762234/the-link-between-exercise-and-titin-passive-stiffness
#12
Sophie Lalande, Patrick J Mueller, Charles S Chung
What is the topic of this review? This review focuses on how in vivo and molecular measurements of cardiac passive stiffness can predict exercise tolerance and how exercise training can reduce cardiac passive stiffness. What advances does it highlight? This review highlights advances in understanding the relationship between molecular (titin-based) and in vivo (left ventricular) passive stiffness, how passive stiffness modifies exercise tolerance, and how exercise training may be therapeutic for cardiac diseases with increased passive stiffness...
September 1, 2017: Experimental Physiology
https://www.readbyqxmd.com/read/28732005/filamin-actin-binding-and-titin-binding-fulfill-distinct-functions-in-z-disc-cohesion
#13
Nicanor González-Morales, Tristan K Holenka, Frieder Schöck
Many proteins contribute to the contractile properties of muscles, most notably myosin thick filaments, which are anchored at the M-line, and actin thin filaments, which are anchored at the Z-discs that border each sarcomere. In humans, mutations in the actin-binding protein Filamin-C result in myopathies, but the underlying molecular function is not well understood. Here we show using Drosophila indirect flight muscle that the filamin ortholog Cheerio in conjunction with the giant elastic protein titin plays a crucial role in keeping thin filaments stably anchored at the Z-disc...
July 2017: PLoS Genetics
https://www.readbyqxmd.com/read/28727760/detection-of-titin-fragments-in-urine-in-response-to-exercise-induced-muscle-damage
#14
Kazue Kanda, Jun Sakuma, Takayuki Akimoto, Yasuo Kawakami, Katsuhiko Suzuki
Many studies have attempted to determine the associations between blood biomarkers and exercise-induced muscle damage. However, poor correlations between the changes in biomarker levels and the magnitude of muscle symptoms have been reported. Recent advances in proteomic tools offer a strategy for the comprehensive analysis of protein expression, which can be used to identify biomarkers. Here, we used a proteomic analysis to identify urinary proteins that appear in response to a calf-raise exercise, including repetitive eccentric muscle contractions, and found that a titin (also known as connectin) N-terminal fragment molecule appears in the urine after eccentric exercise...
2017: PloS One
https://www.readbyqxmd.com/read/28724722/effect-of-directional-pulling-on-mechanical-protein-degradation-by-atp-dependent-proteolytic-machines
#15
Adrian O Olivares, Hema Chandra Kotamarthi, Benjamin J Stein, Robert T Sauer, Tania A Baker
AAA+ proteases and remodeling machines couple hydrolysis of ATP to mechanical unfolding and translocation of proteins following recognition of sequence tags called degrons. Here, we use single-molecule optical trapping to determine the mechanochemistry of two AAA+ proteases, Escherichia coli ClpXP and ClpAP, as they unfold and translocate substrates containing multiple copies of the titin(I27) domain during degradation initiated from the N terminus. Previous studies characterized degradation of related substrates with C-terminal degrons...
July 19, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28717163/titin-mediated-thick-filament-activation-through-a-mechanosensing-mechanism-introduces-sarcomere-length-dependencies-in-mathematical-models-of-rat-trabecula-and-whole-ventricle
#16
Lorenzo Marcucci, Takumi Washio, Toshio Yanagida
Recent experimental evidence in skeletal muscle demonstrated the existence of a thick-filament mechanosensing mechanism, acting as a second regulatory system for muscle contraction, in addition to calcium-mediated thin filament regulation. These two systems cooperate to generate force, but the extent to which their interaction is relevant in physiologically contracting muscle was not yet assessed experimentally. Therefore, we included both regulatory mechanisms in a mathematical model of rat trabecula and whole ventricle...
July 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28716623/a-second-truncation-in-ttn-causes-early-onset-recessive-muscular-dystrophy
#17
Elizabeth Harris, Ana Töpf, Anna Vihola, Anni Evilä, Rita Barresi, Judith Hudson, Peter Hackman, Brian Herron, Daniel MacArthur, Hanns Lochmüller, Kate Bushby, Bjarne Udd, Volker Straub
Mutations in the gene encoding the giant skeletal muscle protein titin are associated with a variety of muscle disorders, including recessive congenital myopathies ±cardiomyopathy, limb girdle muscular dystrophy (LGMD) and late onset dominant distal myopathy. Heterozygous truncating mutations have also been linked to dilated cardiomyopathy. The phenotypic spectrum of titinopathies is emerging and expanding, as next generation sequencing techniques make this large gene amenable to sequencing. We undertook whole exome sequencing in four individuals with LGMD...
June 22, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28712031/human-skeletal-muscle-type-1-fibre-distribution-and-response-of-stress-sensing-proteins-along-the-titin-molecule-after-submaximal-exhaustive-exercise
#18
Satu O A Koskinen, Heikki Kyröläinen, Riina Flink, Harri P Selänne, Sheila S Gagnon, Juha P Ahtiainen, Bradley C Nindl, Maarit Lehti
Early responses of stress-sensing proteins, muscle LIM protein (MLP), ankyrin repeat proteins (Ankrd1/CARP and Ankrd2/Arpp) and muscle-specific RING finger proteins (MuRF1 and MuRF2), along the titin molecule were investigated in the present experiment after submaximal exhaustive exercise. Ten healthy men performed continuous drop jumping unilaterally on a sledge apparatus with a submaximal height until complete exhaustion. Five stress-sensing proteins were analysed by mRNA measurements from biopsies obtained immediately and 3 h after the exercise from exercised vastus lateralis muscle while control biopsies were obtained from non-exercised legs before the exercise...
July 15, 2017: Histochemistry and Cell Biology
https://www.readbyqxmd.com/read/28696205/increasing-evidence-of-mechanical-force-as-a-functional-regulator-in-smooth-muscle-myosin-light-chain-kinase
#19
Fabian Baumann, Magnus Sebastian Bauer, Martin Rees, Alexander Alexandrovich, Mathias Gautel, Diana Angela Pippig, Hermann Eduard Gaub
Mechanosensitive proteins are key players in cytoskeletal remodeling, muscle contraction, cell migration and differentiation processes. Smooth muscle myosin light chain kinase (smMLCK) is a member of a diverse group of serine/threonine kinases that feature cytoskeletal association. Its catalytic activity is triggered by a conformational change upon Ca(2+)/calmodulin (Ca(2+)/CaM) binding. Due to its significant homology with the force-activated titin kinase, smMLCK is suspected to be also regulatable by mechanical stress...
July 11, 2017: ELife
https://www.readbyqxmd.com/read/28676430/insulin-regulates-titin-pre-mrna-splicing-through-the-pi3k-akt-mtor-kinase-axis-in-a-rbm20-dependent-manner
#20
Chaoqun Zhu, Zhiyong Yin, Bihua Tan, Wei Guo
Titin, a giant sarcomeric protein, is largely responsible for the diastolic properties of the heart. It has two major isoforms, N2B and N2BA due to pre-mRNA splicing regulated mainly by a splicing factor RNA binding motif 20 (RBM20). Mis-splicing of titin pre-mRNA in response to external stimuli may lead to altered ratio of N2B to N2BA, and thus, impaired cardiac contractile function. However, little is known about titin alternative splicing in response to external stimuli. Here, we reported the detailed mechanisms of titin alternative splicing in response to insulin...
September 2017: Biochimica et Biophysica Acta
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