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https://www.readbyqxmd.com/read/29778303/the-physiopathologic-interplay-between-stem-cells-and-tissue-niche-in-muscle-regeneration-and-the-role-of-il-6-on-muscle-homeostasis-and-diseases
#1
Laura Forcina, Carmen Miano, Antonio Musarò
Skeletal muscle is a complex, dynamic tissue characterized by an elevated plasticity. Although the adult muscle is mainly composed of multinucleated fibers with post mitotic nuclei, it retains a remarkable ability to regenerate in response to traumatic events. The regenerative potential of the adult skeletal muscle relies in the activity of satellite cells, mononucleated cells residing within the muscle in intimate association with myofibers. Satellite cells normally remain quiescent in their sublaminar position, sporadically entering the cell cycle to guarantee an efficient cellular turnover, by fusing with pre-existing myofibers, and to maintain the stem cell pool...
May 16, 2018: Cytokine & Growth Factor Reviews
https://www.readbyqxmd.com/read/29778277/interpretation-of-acid-%C3%AE-glucosidase-activity-in-creatine-kinase-elevation-a-case-of-becker-muscular-dystrophy
#2
Yoshiki Oitani, Akihiko Ishiyama, Motomichi Kosuga, Kentaro Iwasawa, Ayako Ogata, Fumiko Tanaka, Eri Takeshita, Yuko Shimizu-Motohashi, Hirofumi Komaki, Ichizo Nishino, Torayuki Okuyama, Masayuki Sasaki
BACKGROUND: Diagnosis of Pompe disease is sometimes challenging because it exhibits clinical similarities to muscular dystrophy. CASE: We describe a case of Becker muscular dystrophy (BMD) with a remarkable reduction in activity of the acid α-glucosidase (GAA) enzyme, caused by a combination of pathogenic mutation and polymorphism variants resulting in pseudodeficiency in GAA. The three-year-old boy demonstrated asymptomatic creatine kinase elevation. Neither exon deletion nor duplication was detected on multiplex ligation-dependent probe amplification (MLPA) of DMD...
May 16, 2018: Brain & Development
https://www.readbyqxmd.com/read/29776992/cardiomyopathy-mutation-f88l-in-troponin-t-abolishes-length-dependency-of-myofilament-ca-2-sensitivity
#3
Sherif M Reda, Murali Chandra
Recent clinical studies have revealed a new hypertrophic cardiomyopathy-associated mutation (F87L) in the central region of human cardiac troponin T (TnT). However, despite its implication in several incidences of sudden cardiac death in young and old adults, whether F87L is associated with cardiac contractile dysfunction is unknown. Because the central region of TnT is important for modulating the muscle length-mediated recruitment of new force-bearing cross-bridges (XBs), we hypothesize that the F87L mutation causes molecular changes that are linked to the length-dependent activation of cardiac myofilaments...
May 18, 2018: Journal of General Physiology
https://www.readbyqxmd.com/read/29775754/effects-of-micrornas-on-skeletal-muscle-development
#4
REVIEW
J Wang, L Z Yang, J S Zhang, J X Gong, Y H Wang, C L Zhang, H Chen, X T Fang
MicroRNAs (miRNAs) are small (about 22 nucleotides) noncoding RNAs, which were highly conserved among mammals. They have ushered in a new era in molecular biology over twenty years. They can negatively regulate gene expression at the posttranscriptional level through the principle of complementary base pairing with the 3' untranslated region (UTR) of their target mRNAs and induce their degradation. They involve in tissue morphogenesis, cellular processes like apoptosis, and major signaling pathways. Previous studies have promoted our understanding that miRNAs play an important role in myogenesis and have a big impact on muscle mass, muscle fiber type and muscle diseases...
May 15, 2018: Gene
https://www.readbyqxmd.com/read/29775750/the-effects-of-capn1-gene-inactivation-on-the-differential-expression-of-genes-in-skeletal-muscle
#5
William T Olive, Brittney N Keel, Amanda K Lindholm-Perry, Justyna Horodyska, Andrew P Foote
Protein turnover is required for muscle growth and regeneration and several proteolytic enzymes, including the calpains, degrade myofibrillar proteins during this process. In a previous experiment, phenotypic differences were observed between μ-calpain knockout (KO) and wild type (WT) mice, including nutrient accretion and fiber type differences. These changes were particularly evident as the animals aged. Thus, we utilized 18 mice (9 KO and 9 WT) to compare transcript abundance to identify differentially expressed genes (DEGs) at 52 wk of age...
May 15, 2018: Gene
https://www.readbyqxmd.com/read/29775504/biomimetic-supramolecular-fibers-exhibit-water-induced-supercontraction
#6
Yuchao Wu, Darshil U Shah, Baoyuan Wang, Ji Liu, Xiaohe Ren, Michael H Ramage, Oren A Scherman
Spider silk is a fascinating material, combining high strength and elasticity that outperforms most synthetic fibers. Another intriguing feature of spider silk is its ability to "supercontract," shrinking up to 50% when exposed to water. This is likely on account of the entropy-driven recoiling of secondary structured proteins when water penetrates the spider silk. In contrast, humidity-driven contraction in synthetic fibers is difficult to achieve. Here, inspired by the spider silk model, a supercontractile fiber (SCF), which contracts up to 50% of its original length at high humidity, comparable to spider silk, is reported...
May 18, 2018: Advanced Materials
https://www.readbyqxmd.com/read/29774991/aggregate-mesenchymal-stem-cell-delivery-ameliorates-the-regenerative-niche-for-muscle-repair
#7
Marissa A Ruehle, Hazel Y Stevens, Aaron M Beedle, Robert E Guldberg, Jarrod A Call
Duchenne muscular dystrophy (DMD) is a severe muscle wasting disease due to the absence of the dystrophin protein from the muscle cell membrane which renders the muscle susceptible to continuous damage. In DMD patients, muscle weakness, together with cycles of degeneration/regeneration and replacement with non-contractile tissue, limit mobility and lifespan. Since the loss of dystrophin result in loss of polarity and a reduction in the number of self-renewing satellite cells, it is postulated that these patients could achieve an improved quality of life if delivered cells could restore satellite cell function...
May 18, 2018: Journal of Tissue Engineering and Regenerative Medicine
https://www.readbyqxmd.com/read/29774231/semaphorin3a-signaling-is-dispensable-for-motor-axon-reinnervation-of-the-adult-neuromuscular-junction
#8
Jennifer L Shadrach, Brian A Pierchala
The neuromuscular junction (NMJ) is a specialized synapse that is formed by motor axon innervation of skeletal muscle fibers. The maintenance of motor-muscle connectivity is critical for the preservation of muscle tone and generation of movement. Injury can induce a robust regenerative response in motor axons, but severe trauma or chronic denervation resulting from neurodegenerative disease typically leads to inefficient repair and poor functional recovery. The axon guidance molecule Semaphorin3A (Sema3A) has been implicated as a negative regulator of motor innervation...
May 2018: ENeuro
https://www.readbyqxmd.com/read/29773865/perlecan-a-heparan-sulfate-proteoglycan-regulates-systemic-metabolism-with-dynamic-changes-in-adipose-tissue-and-skeletal-muscle
#9
Yuri Yamashita, Satoshi Nakada, Toshinori Yoshihara, Takeshi Nara, Norihiko Furuya, Takashi Miida, Nobutaka Hattori, Eri Arikawa-Hirasawa
Perlecan (HSPG2), a heparan sulfate proteoglycan, is a component of basement membranes and participates in a variety of biological activities. Here, we show physiological roles of perlecan in both obesity and the onset of metabolic syndrome. The perinatal lethality-rescued perlecan knockout (Hspg2-/- -Tg) mice showed a smaller mass and cell size of white adipose tissues than control (WT-Tg) mice. Abnormal lipid deposition, such as fatty liver, was not detected in the Hspg2-/- -Tg mice, and those mice also consumed more fat as an energy source, likely due to their activated fatty acid oxidation...
May 17, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29772956/lumbar-muscle-structure-predicts-operational-postures-in-active-duty-marines
#10
David B Berry, Bahar Shahidi, Ana E Rodríguez-Soto, Jan M Hughes-Austin, Karen R Kelly, Samuel R Ward
Study Design Cross-sectional. Background The relationship between lumbar spine posture and muscle structure is not well understood. Objectives Investigate the predictive capacity of muscle structure on lumbar spine posture in active duty Marines. Methods Fouty-three Marines were scanned using an upright magnetic resonance imaging (MRI) scanner while standing unloaded and standing, sitting, and prone on elbows with body armor. Cobb, horizontal, and sacral angles were measured. Marines were then scanned unloaded in supine using a supine MRI scanner...
May 17, 2018: Journal of Orthopaedic and Sports Physical Therapy
https://www.readbyqxmd.com/read/29772844/leucine-supplementation-does-not-attenuate-skeletal-muscle-loss-during-leg-immobilization-in-healthy-young-men
#11
Evelien M P Backx, Astrid M H Horstman, Gabriel N Marzuca-Nassr, Janneau van Kranenburg, Joey S Smeets, Cas J Fuchs, Anniek A W Janssen, Lisette C P G M de Groot, Tim Snijders, Lex B Verdijk, Luc J C van Loon
BACKGROUND: Short successive periods of physical inactivity occur throughout life and contribute considerably to the age-related loss of skeletal muscle mass. The maintenance of muscle mass during brief periods of disuse is required to prevent functional decline and maintain metabolic health. OBJECTIVE: To assess whether daily leucine supplementation during a short period of disuse can attenuate subsequent muscle loss in vivo in humans. METHODS: Thirty healthy (22 ± 1 y) young males were exposed to a 7-day unilateral knee immobilization intervention by means of a full leg cast with (LEU, n = 15) or without (CON, n = 15) daily leucine supplementation (2...
May 17, 2018: Nutrients
https://www.readbyqxmd.com/read/29771823/residual-force-enhancement-is-attenuated-in-a-shortening-magnitude-dependent-manner
#12
Atsuki Fukutani, Walter Herzog
INTRODUCTION: The isometric force attained after active stretch is greater than that attained in a purely isometric contraction. This property is referred to as residual force enhancement (RFE). Although RFE is thought to contribute to the enhanced force and power in stretch-shortening cycles (SSCs), it is unclear whether shortening that occurs after active stretch eliminates the RFE induced by active stretch. Therefore, we evaluated the influence of shortening on RFE. METHODS: Skinned rabbit soleus fibers (N = 43) were used for all tests...
May 16, 2018: Medicine and Science in Sports and Exercise
https://www.readbyqxmd.com/read/29771332/mechanisms-of-skeletal-muscle-wasting-in-a-mouse-model-for-myotonic-dystrophy-type-1
#13
Ginny R Morriss, Kimal Rajapakshe, Shixia Huang, Cristian Coarfa, Thomas A Cooper
Myotonic dystrophy type 1 (DM1) is a multisystemic disease resulting in severe muscle weakening and wasting. DM1 is caused by expansion of CTG repeats in the 3'-UTR of the DMPK gene. We have developed an inducible, skeletal muscle-specific mouse model of DM1 (CUG960) that expresses 960 CUG repeats in the context of human DMPK exons 11-15. CUG960 RNA-expressing mice induced at PN1, as well as adult-onset animals, show clear, measurable muscle wasting accompanied by severe histological defects including central myonuclei, reduced fiber cross sectional area, increased percentage of oxidative myofibers, and the presence of nuclear RNA foci that colocalize with Mbnl1 protein...
May 16, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29771049/-a-case-report-of-tracheotomy-complicated-by-hemorrhage-after-radical-operation-of-tongue
#14
M Y Sun, H J Wu
A 55-year old middle-aged male presented swelling lymph nodes in neck and mass in left tongue for three mouths. Physical examination shows a size 5 cm×5 cm mass in tongue and went over the midline. The mandibular lymph nodes were touched with moderate tenderness and little mobility in bilateral. The laboratory results, function of the kidney and heart liver, nasopharyngeal fiber area were basically normal. The result of CT and MRI shows the mass reached the lingual muscle layer and several suspected metastatic lymph nodes in neck...
May 20, 2017: Journal of Clinical Otorhinolaryngology, Head, and Neck Surgery
https://www.readbyqxmd.com/read/29770361/differential-diagnosis-of-vacuolar-muscle-biopsies-use-of-p62-lc3-and-lamp2-immunohistochemistry
#15
Elisa Vittonatto, Silvia Boschi, Loredana CHIADò-Piat, Valentina Ponzalino, Sara Bortolani, Chiara Brusa, Innocenzo Rainero, Federica Ricci, Liliana Vercelli, Tiziana Mongini
Intrafibral vacuoles are the morphological hallmark in a wide variety of human skeletal muscle disorders with different etiology. In most cases, differential diagnosis is feasible with a routine histochemical work up of muscle biopsy. Ultrastructural analysis is an important confirmatory tool, but it is not widely available. Immunohistochemical stainings for p62, LAMP2 and LC3 are commonly available as tissutal marker for autophagy. We compared the immunohistochemical patterns for autophagic markers p62, LC3 and LAMP2 with routine histochemical markers in 39 biopsies from patients with definite diagnoses of glycogen storage disease type 2 (LOPD or Pompe disease, PD), sporadic inclusion body myositis (sIBM), oculo-pharyngeal muscular dystrophy (OPMD) and necrotizing myopathy (NM)...
December 2017: Acta Myologica: Myopathies and Cardiomyopathies: Official Journal of the Mediterranean Society of Myology
https://www.readbyqxmd.com/read/29768191/loss-of-a-negative-regulator-of-mtorc1-induces-aerobic-glycolysis-and-altered-fiber-composition-in-skeletal-muscle
#16
Paul A Dutchak, Sandi J Estill-Terpack, Abigail A Plec, Xiaozheng Zhao, Chendong Yang, Jun Chen, Bookyung Ko, Ralph J Deberardinis, Yonghao Yu, Benjamin P Tu
The conserved GATOR1 complex consisting of NPRL2-NPRL3-DEPDC5 inhibits mammalian target of rapamycin complex 1 (mTORC1) in response to amino acid insufficiency. Here, we show that loss of NPRL2 and GATOR1 function in skeletal muscle causes constitutive activation of mTORC1 signaling in the fed and fasted states. Muscle fibers of NPRL2 knockout animals are significantly larger and show altered fiber-type composition, with more fast-twitch glycolytic and fewer slow-twitch oxidative fibers. NPRL2 muscle knockout mice also have altered running behavior and enhanced glucose tolerance...
May 15, 2018: Cell Reports
https://www.readbyqxmd.com/read/29768017/the-signaling-network-resulting-in-ventilator-induced-diaphragm-dysfunction
#17
Huibin Tang, Joseph B Shrager
Mechanical ventilation (MV) is a life-saving measure for those incapable of adequately ventilating or oxygenating without assistance. Unfortunately, even brief periods of MV result in diaphragm weakness (i.e., "ventilator-induced diaphragm dysfunction" - VIDD) that may render it difficult to wean the ventilator. Prolonged MV is associated with cascading complications and is a strong risk factor for death. Thus, prevention of VIDD may have a dramatic impact on mortality rates. Here, we summarized the current understanding of the pathogenic events underlying VIDD...
May 16, 2018: American Journal of Respiratory Cell and Molecular Biology
https://www.readbyqxmd.com/read/29766941/idiopathic-inflammatory-myopathies-in-adults-a-comparative-study-of-bohan-and-peter-and-european-neuromuscular-center-2004-criteria
#18
Sundaram Challa, Saumya Jakati, Megha S Uppin, Meena A Kannan, Rajasekhar Liza, M K Murthy Jagarlapudi
Background: Bohan and Peter criteria are widely used for the diagnosis of idiopathic inflammatory myopathies (IIMs). Recently, European Neuromuscular Center (ENMC) formulated criteria to identify subgroups of IIMs. Aim: To compare the two diagnostic criteria in adult IIMs. Materials and Methods: This was a retrospective review of case records of histologically confirmed IIMs in adults between January 2014 and May 2015. Both the Bohan and Peter, and ENMC 2004 criteria were applied in the same group of patients to subgroup the IIMs...
May 2018: Neurology India
https://www.readbyqxmd.com/read/29764991/a-dgk%C3%AE-foxo-ubiquitin-proteolytic-axis-controls-fiber-size-during-skeletal-muscle-remodeling
#19
Jae-Sung You, Matthew S Dooley, Chan-Ran Kim, Eui-Jun Kim, Wei Xu, Craig A Goodman, Troy A Hornberger
Skeletal muscle rapidly remodels in response to various stresses, and the resulting changes in muscle mass profoundly influence our health and quality of life. We identified a diacylglycerol kinase ζ (DGKζ)-mediated pathway that regulated muscle mass during remodeling. During mechanical overload, DGKζ abundance was increased and required for effective hypertrophy. DGKζ not only augmented anabolic responses but also suppressed ubiquitin-proteasome system (UPS)-dependent proteolysis. We found that DGKζ inhibited the transcription factor FoxO that promotes the induction of the UPS...
May 15, 2018: Science Signaling
https://www.readbyqxmd.com/read/29763467/natural-disease-history-of-the-dy2j-mouse-model-of-laminin-%C3%AE-2-merosin-deficient-congenital-muscular-dystrophy
#20
S Pasteuning-Vuhman, K Putker, C L Tanganyika-de Winter, J W Boertje-van der Meulen, L van Vliet, M Overzier, J J Plomp, A Aartsma-Rus, M van Putten
Merosin deficient congenital muscular dystrophy 1A (MDC1A) is a very rare autosomal recessive disorder caused by mutations in the LAMA2 gene leading to severe and progressive muscle weakness and atrophy. Although over 350 causative mutations have been identified for MDC1A, no treatment is yet available. There are many therapeutic approaches in development, but the lack of natural history data of the mouse model and standardized outcome measures makes it difficult to transit these pre-clinical findings to clinical trials...
2018: PloS One
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