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Muscular chains

Hitoshi Suzuki, Yoshitsugu Aoki, Toshiki Kameyama, Takashi Saito, Satoru Masuda, Jun Tanihata, Tetsuya Nagata, Akila Mayeda, Shin'ichi Takeda, Toshifumi Tsukahara
Duchenne muscular dystrophy (DMD) is a severe muscular disorder. It was reported that multiple exon skipping (MES), targeting exon 45-55 of the DMD gene, might improve patients' symptoms because patients who have a genomic deletion of all these exons showed very mild symptoms. Thus, exon 45-55 skipping treatments for DMD have been proposed as a potential clinical cure. Herein, we detected the expression of endogenous exons 44-56 connected mRNA transcript of the DMD using total RNAs derived from human normal skeletal muscle by reverse transcription polymerase chain reaction (RT-PCR), and identified a total of eight types of MES products around the hotspot...
October 13, 2016: International Journal of Molecular Sciences
Yin Chen, Megan Reddy, Yanlei Yu, Fuming Zhang, Robert J Linhardt
Glycosaminoglycans (GAGs) were prepared from the muscular stomach or gizzard of the chicken. The content of GAGs on a dry weight basis contains 0.4 wt.% a typical value observed for a muscle tissue. The major GAG components were chondroitin-6-sulfate and chondroitin-4-sulfate (~64 %) of molecular weight 21-22 kDa. Hyaluronan (~24 %) had a molecular weight 120 kDa. Smaller amounts (12 %) of heparan sulfate was also present which was made of more highly sulfated chains of molecular weight of 21-22 kDa and a less sulfated low molecular weight (< 10 kDa) heterogeneous partially degraded heparan sulfate...
October 17, 2016: Glycoconjugate Journal
Hadas Newman, Sergiu C Blumen, Itzhak Braverman, Rana Hanna, Beatrice Tiosano, Ido Perlman, Tamar Ben-Yosef
Purpose: To investigate the genetic basis for severe visual complaints by Bukharan Jewish patients with oculopharyngeal muscular dystrophy (OPMD). Methods: Polymerase chain reaction amplification and direct sequencing were used to test for NRL, PABPN1, and NR2E3 mutations. Complete ophthalmic examination included best-corrected visual acuity, biomicroscopic examination, optical coherence tomography, and fundus autofluorescence. Detailed electroretinography (ERG) testing was conducted including expanded International Society for Clinical Electrophysiology of Vision protocol for light-adapted and dark-adapted conditions, measurements of S-cone function, and ON-OFF light-adapted ERG...
October 1, 2016: Investigative Ophthalmology & Visual Science
Eun Ji Ahn, Mi Sun Yum, Eun Hee Kim, Han Wook Yoo, Beom Hee Lee, Gu Hwan Kim, Tae Sung Ko
BACKGROUND AND PURPOSE: Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disease characterized by progressive muscle weakness and atrophy. Most SMA patients have a homozygous deletion in survival of motor neuron 1 (SMN1) gene, and neuronal apoptosis inhibitory protein (NAIP) gene is considered a phenotype modifier. We investigated the genotype-phenotype correlation of SMN1 and NAIP deletions in Korean SMA patients. METHODS: Thirty-three patients (12 males and 21 females) treated at the Asan Medical Center between 1999 and 2013 were analyzed retrospectively...
October 7, 2016: Journal of Clinical Neurology
Abderahim Gaceb, Luisa Vergori, M C Martinez, Ramaroson Andriantsitohaina
Microvesicles, small membrane vesicles released from cells, have beneficial and/or deleterious effects in sepsis. We previously reported that non-muscle myosin light chain kinase (nmMLCK) deletion protects mice against endotoxic shock by reducing inflammation. Here, we have evaluated the consequences of nmMLCK deletion on microvesicle phenotypes and their effects on mouse aortic endothelial cells in association with vascular inflammation and endothelial dysfunction during endotoxic shock induced by lipopolysaccharide in mice...
2016: Frontiers in Pharmacology
Ute Ulrike Botzenhart, Constantin Wegenstein, Teodor Todorov, Christiane Kunert-Keil
The most widespread animal model to investigate Duchenne muscular dystrophy is the mdx-mouse. In contrast to humans, phases of muscle degeneration are replaced by regeneration processes; hence there is only a restricted time slot for research. The aim of the study was to investigate if an intramuscular injection of BTX-A is able to break down muscle regeneration and has direct implications on the gene expression of myosin heavy chains in the corresponding treated and untreated muscles. Therefore, paralysis of the right masseter muscle was induced in adult healthy and dystrophic mice by a specific intramuscular injection of BTX-A...
2016: BioMed Research International
Xiaoxi Zhu, Melanie Kny, Franziska Schmidt, Alexander Hahn, Tobias Wollersheim, Christian Kleber, Steffen Weber-Carstens, Jens Fielitz
OBJECTIVE: In sepsis, the disease course of critically ill patients is often complicated by muscle failure leading to ICU-acquired weakness. The myokine transforming growth factor-β1 increases during inflammation and mediates muscle atrophy in vivo. We observed that the transforming growth factor-β1 inhibitor, secreted frizzled-related protein 2, was down-regulated in skeletal muscle of ICU-acquired weakness patients. We hypothesized that secreted frizzled-related protein 2 reduction enhances transforming growth factor-β1-mediated effects and investigated the interrelationship between transforming growth factor-β1 and secreted frizzled-related protein 2 in inflammation-induced atrophy...
September 21, 2016: Critical Care Medicine
Toshiaki Takahashi, Florian Friedmacher, Julia Zimmer, Prem Puri
PURPOSE: Congenital diaphragmatic hernia (CDH) is presumed to originate from defects in the primordial diaphragmatic mesenchyme, mainly comprising of muscle connective tissue (MCT). Thus, normal diaphragmatic morphogenesis depends on the structural integrity of the underlying MCT. Developmental mutations that inhibit normal formation of diaphragmatic MCT have been shown to result in CDH. Desmin (DES) is a major filament protein in the MCT, which is essential for the tensile strength of the developing diaphragm muscle...
September 20, 2016: Pediatric Surgery International
S Lataoui, S Belghali, H Zeglaoui, E Bouajina, H Ben Saad
BACKGROUND: Studies about sub-maximal aerobic capacity of patients with rheumatoid arthritis are scarce. AIMS: To assess the sub-maximal aerobic capacity of these patients through the 6-min walk test, estimated age of the "muscular and cardiorespiratory" chain. METHODS: Thirty-seven consecutive patients (aged 20 to 60 years) with newly diagnosed rheumatoid arthritis will be included. Non-inclusion criteria will be: use of drugs (e.g.; methotrexate, beta-blockers), orthopaedic or rheumatologic conditions (other than rheumatoid arthritis) that may alter walking ability and recent infections...
September 14, 2016: Revue des Maladies Respiratoires
Thomas Fröhlich, Elisabeth Kemter, Florian Flenkenthaler, Nikolai Klymiuk, Kathrin A Otte, Andreas Blutke, Sabine Krause, Maggie C Walter, Rüdiger Wanke, Eckhard Wolf, Georg J Arnold
Duchenne muscular dystrophy (DMD) is caused by genetic deficiency of dystrophin and characterized by massive structural and functional changes of skeletal muscle tissue, leading to terminal muscle failure. We recently generated a novel genetically engineered pig model reflecting pathological hallmarks of human DMD better than the widely used mdx mouse. To get insight into the hierarchy of molecular derangements during DMD progression, we performed a proteome analysis of biceps femoris muscle samples from 2-day-old and 3-month-old DMD and wild-type (WT) pigs...
2016: Scientific Reports
Zhi-Dong Zhou, Wuan-Ting Saw, Eng-King Tan
The coiled-coil-helix-coiled-coil-helix domain (CHCHD)-containing proteins are evolutionarily conserved nucleus-encoded small mitochondrial proteins with important functions. So far, nine members have been identified in this protein family. All CHCHD proteins have at least one functional coiled-coil-helix-coiled-coil-helix (CHCH) domain, which is stabilized by two pairs of disulfide bonds between two helices. CHCHD proteins have various important pathophysiological roles in mitochondria and other key cellular processes...
September 8, 2016: Molecular Neurobiology
Charles P Gabel, Natalie Rando, Markus Melloh
To ascertain the effectiveness of slacklining as a supplementary therapy for elderly stroke patients who are functionally non-progressing. This case study involved an 18-mo prospective observation of the management of an 87-year-old female stroke-patient of the left hemisphere with reduced balance, reduced lower limb muscular activation, hypertonia, and concurrent postural deficits. This entailed the initial acute care phase through to discharge to home and 18-mo final status in her original independent living setting...
August 18, 2016: World Journal of Orthopedics
Xueying Su, Ruizhu Lin, Yonglan Huang, Huiying Sheng, Xiaofei Li, Tzer Hwu Ting, Li Liu, Xiuzhen Li
OBJECTIVE: To investigate the clinical and molecular features of three Chinese patients with various typical manifestations of congenital generalized lipodystrophy (CGL). METHODS: Clinical symptoms, results of laboratory analyses, and data on previous treatments in three Chinese patients were collected by retrospective review of medical records. All coding regions and adjacent exon-intron junction regions of AGPAT2 and BSCL2 genes were amplified by polymerase chain reaction and sequenced...
September 9, 2016: Journal of Clinical Research in Pediatric Endocrinology
M P Ellies-Oury, R Dumont, G Perrier, M Roux, D Micol, B Picard
In practice cattle may be slaughtered at different combinations of age and weight. As each of these factors could affect meat quality traits, the present work aimed to identify which combination can be expected to increase overall meat quality of m. rectus abdominis of Charolais heifers. Totally, 40 heifers were slaughtered either at 26±1 or at 36±1 months of age. Young heifers were sampled at two different carcass weights (349±12 and 394±8 kg). Old heifers were also sampled at two different carcass weights (397±6 and 451±9 kg)...
September 9, 2016: Animal: An International Journal of Animal Bioscience
Sara Oliván, Ana C Calvo, Amaya Rando, Mireia Herrando-Grabulosa, Raquel Manzano, Pilar Zaragoza, Eduardo F Tizzano, Jose Aquilera, Rosario Osta
Spinal muscular atrophy (SMA) is a hereditary childhood disease that causes paralysis and progressive degeneration of skeletal muscles and spinal motor neurons. SMA is associated with reduced levels of full-length Survival of Motor Neuron (SMN) protein, due to mutations in the Survival of Motor Neuron 1 gene. Nowadays there are no effective therapies available to treat patients with SMA, so our aim was to test whether the non-toxic carboxy-terminal fragment of tetanus toxin heavy chain (TTC), which exhibits neurotrophic properties, might have a therapeutic role or benefit in SMA...
2016: Frontiers in Molecular Neuroscience
Yinghui Li, Fengna Li, Li Wu, Hongkui Wei, Yingying Liu, Tiejun Li, Bie Tan, Xiangfeng Kong, Kang Yao, Shuai Chen, Fei Wu, Yehui Duan, Yulong Yin
BACKGROUND: To investigate the effects of dietary crude protein (CP) restriction on muscle fiber characteristics and key regulators related to protein deposition in skeletal muscle, a total of 18 growing-finishing pigs (62.30 ± 0.88 kg) were allotted to 3 groups and fed with the recommended adequate protein (AP, 16 % CP) diet, moderately restricted protein (MP, 13 % CP) diet and low protein (LP, 10 % CP) diet, respectively. The skeletal muscle of different locations in pigs, including longissimus dorsi muscle (LDM), psoas major muscle (PMM) and biceps femoris muscle (BFM) were collected and analyzed...
2016: Journal of Animal Science and Biotechnology
Dörte Lodka, Aanchal Pahuja, Cornelia Geers-Knörr, Renate J Scheibe, Marcel Nowak, Jida Hamati, Clemens Köhncke, Bettina Purfürst, Tamara Kanashova, Sibylle Schmidt, David J Glass, Ingo Morano, Arnd Heuser, Theresia Kraft, Rhonda Bassel-Duby, Eric N Olson, Gunnar Dittmar, Thomas Sommer, Jens Fielitz
BACKGROUND: The Muscle-specific RING-finger (MuRF) protein family of E3 ubiquitin ligases is important for maintenance of muscular structure and function. MuRF proteins mediate adaptation of striated muscles to stress. MuRF2 and MuRF3 bind to microtubules and are implicated in sarcomere formation with noticeable functional redundancy. However, if this redundancy is important for muscle function in vivo is unknown. Our objective was to investigate cooperative function of MuRF2 and MuRF3 in the skeletal muscle and the heart in vivo...
May 2016: Journal of Cachexia, Sarcopenia and Muscle
Yin-Liang Lin, Andrew Karduna
Proprioception is essential for shoulder neuromuscular control and shoulder stability. Exercise of the rotator cuff and scapulothoracic muscles is an important part of shoulder rehabilitation. The purpose of this study was to investigate the effect of rotator cuff and scapulothoracic muscle exercises on shoulder joint position sense. Thirty-six healthy subjects were recruited and randomly assigned into either a control or training group. The subjects in the training group received closed-chain and open-chain exercises focusing on rotator cuff and scapulothoracic muscles for four weeks...
October 2016: Human Movement Science
Teppei Ikeda, Osamu Ichii, Saori Otsuka-Kanazawa, Teppei Nakamura, Yaser Hosny Ali Elewa, Yasuhiro Kon
Skeletal muscle myofibers constantly undergo degeneration and regeneration. Histopathological features of 6 skeletal muscles (cranial tibial [CT], gastrocnemius, quadriceps femoris, triceps brachii [TB], lumbar longissimus muscles, and costal part of the diaphragm [CPD]) were compared using C57BL/10ScSn-Dmd (mdx) (mdx) mice, a model for muscular dystrophy versus control, C57BL/10 mice. Body weight and skeletal muscle mass were lower in mdx mice than the control at 4 weeks of age; these results were similar at 6-30 weeks...
July 29, 2016: Journal of Muscle Research and Cell Motility
Wesley C Kephart, Petey W Mumford, Anna E McCloskey, A Maleah Holland, Joshua J Shake, C Brooks Mobley, Adam E Jagodinsky, Wendi H Weimar, Gretchen D Oliver, Kaelin C Young, Jordan R Moon, Michael D Roberts
BACKGROUND: Amino acid supplementation has been shown to potentially reduced exercise-induced muscle soreness. Thus, the purpose of this study was to examine if branched chain amino acid and carbohydrate (BCAACHO) versus carbohydrate-only sports drink (CHO) supplementation attenuated markers of muscle damage while preserving performance markers following 3 days of intense weight training. METHODS: Healthy resistance-trained males (n = 30) performed preliminary testing (T1) whereby they: 1) donated a baseline blood draw, 2) performed knee extensor dynamometry to obtain peak quadriceps isometric and isokinetic torque as well as electromyography (EMG) activity at 60°/s and 120°/s, and 3) performed a one repetition maximum (1RM) barbell back squat...
2016: Journal of the International Society of Sports Nutrition
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