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https://www.readbyqxmd.com/read/28222529/progressive-motor-deficit-is-mediated-by-the-denervation-of-neuromuscular-junctions-and-axonal-degeneration-in-transgenic-mice-expressing-mutant-p301s-tau-protein
#1
Zhuoran Yin, Femke Valkenburg, Betty E Hornix, Ietje Mantingh-Otter, Xingdong Zhou, Muriel Mari, Fulvio Reggiori, Debby Van Dam, Bart J L Eggen, Peter P De Deyn, Erik Boddeke
Tauopathies include a variety of neurodegenerative diseases associated with the pathological aggregation of hyperphosphorylated tau, resulting in progressive cognitive decline and motor impairment. The underlying mechanism for motor deficits related to tauopathy is not yet fully understood. Here, we use a novel transgenic tau mouse line, Tau 58/4, with enhanced neuron-specific expression of P301S mutant tau to investigate the motor abnormalities in association with the peripheral nervous system. Using stationary beam, gait, and rotarod tests, motor deficits were found in Tau 58/4 mice already 3 months after birth, which deteriorated during aging...
February 10, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28222366/alterations-of-tissue-metallothionein-and-vitellogenin-concentrations-in-tropical-cup-oysters-saccostrea-sp-following-short-term-96h-exposure-to-cadmium
#2
Angela M Moncaleano-Niño, Sergio A Barrios-Latorre, Javier F Poloche-Hernández, Vanessa Becquet, Valérie Huet, Luisa Villamil, Hélène Thomas-Guyon, Michael J Ahrens, Andrea Luna-Acosta
Metallothioneins and vitellogenins are low molecular weight proteins that have been used widely in environmental monitoring as biomarkers of exposure and damage to metals and estrogenic compounds, respectively. In the present study, the responses of metallothionein and vitellogenin tissue concentrations were measured following acute (96h) aqueous exposures to cadmium in Saccostrea sp., a tropical cup oyster native to the Western Pacific Ocean that has recently established itself in the Caribbean Sea. Adult oysters (1...
February 9, 2017: Aquatic Toxicology
https://www.readbyqxmd.com/read/28221913/an-immunoassay-for-quantification-of-contamination-by-raw-meat-juice-on-food-contact-surfaces
#3
Fur-Chi Chen, Sandria Godwin, Edgar Chambers
Raw chicken products often are contaminated with Salmonella and Campylobacter , which can be transmitted from packages to contact surfaces. Raw meat juices from these packages also provide potential media for cross-contamination. There are limited quantitative data on the levels of consumer exposure to raw meat juice during shopping for and handling of chicken products. An exposure assessment is needed to quantify the levels of transmission and to assess the risk. An enzyme-linked immunosorbent assay (ELISA) was developed and validated for quantitative detection of raw meat juice on hands and various food contact surfaces...
November 2016: Journal of Food Protection
https://www.readbyqxmd.com/read/28221306/whole-exome-sequencing-identifies-atypical-welander-distal-myopathy-in-patient
#4
Jennifer Gass, Patrick Blackburn, Jessica Jackson, Kimberly Harris, Duygu Selcen, Elliot Dimberg, Paldeep Atwal
Welander distal myopathy is a rare autosomal dominant disorder characterized by muscle weakness in the hands and feet. Exome sequencing of affected families discovered a segregating p.Glu384Lys pathogenic variant in TIA-1 as the main genetic cause of Welander distal myopathy. TIA-1 encodes an RNA-binding protein which serves as a key component of stress granules. This protein also regulates splicing and translation of mRNA. Our patient developed progressive weakness in his hands and feet during his late 40s that was misdiagnosed as a neuropathy that caused muscle atrophy...
March 2017: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/28220527/recessive-mypn-mutations-cause-cap-myopathy-with-occasional-nemaline-rods
#5
Xavière Lornage, Edoardo Malfatti, Chrystel Chéraud, Raphaël Schneider, Valérie Biancalana, Jean-Marie Cuisset, Matteo Garibaldi, Bruno Eymard, Michel Fardeau, Anne Boland, Jean-François Deleuze, Julie Thompson, Robert-Yves Carlier, Johann Böhm, Norma B Romero, Jocelyn Laporte
Congenital myopathies are phenotypically and genetically heterogeneous. We describe homozygous truncating mutations in MYPN in 2 unrelated families with a slowly progressive congenital cap myopathy. MYPN encodes the Z-line protein myopalladin implicated in sarcomere integrity. Functional experiments demonstrate that the mutations lead to mRNA defects and to a strong reduction in full-length protein expression. Myopalladin signals accumulate in the caps together with alpha-actinin. Dominant MYPN mutations were previously reported in cardiomyopathies...
February 21, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28220374/lipopolysaccharide-activates-toll-like-receptor-4-and-prevents-cardiac-fibroblast-to-myofibroblast-differentiation
#6
Samir Bolívar, Roxana Santana, Pedro Ayala, Rodolfo Landaeta, Pía Boza, Claudio Humeres, Raúl Vivar, Claudia Muñoz, Viviana Pardo, Samuel Fernandez, Renatto Anfossi, Guillermo Diaz-Araya
Bacterial lipopolysaccharide (LPS) is a known ligand of Toll-like receptor 4 (TLR4) which is expressed in cardiac fibroblasts (CF). Differentiation of CF to cardiac myofibroblasts (CMF) is induced by transforming growth factor-β1 (TGF-β1), increasing alpha-smooth muscle actin (α-SMA) expression. In endothelial cells, an antagonist effect between LPS-induced signaling and canonical TGF-β1 signaling was described; however, it has not been studied whether in CF and CMF the expression of α-SMA induced by TGF-β1 is antagonized by LPS and the mechanism involved...
February 20, 2017: Cardiovascular Toxicology
https://www.readbyqxmd.com/read/28220339/triphenyltin-hydroxide-induces-changes-in-the-oxidative-stress-parameters-of-fish
#7
Bárbara Clasen, Alexssandro G Becker, Thais Lópes, Camila R Murussi, Fabiane G Antes, Roberta C Horn, Érico M M Flores, Bernardo Baldisserotto, Valderi L Dressler, Vania L Loro
Among all organotin compounds , triphenyltin hydroxide (TPhTH) is widely used as fungicide and moluscicide in Brazil. However, the effects of TPhTH on the biochemical parameters of non-target organisms, such as fish, are little known. The aim of the present study is to assess the possible toxic effects of different concentrations of waterborne TPhTH on silver catfish belonging to species Rhamdia quelen. The fish were exposed to two different concentrations of TPhTH (1.08 and 1.70 µg/L as Sn) for 15 days and then compared to the control group (triplicate, n = 3)...
February 20, 2017: Ecotoxicology
https://www.readbyqxmd.com/read/28220207/considerations-on-mtor-regulation-at-serine-2448-implications-for-muscle-metabolism-studies
#8
REVIEW
Vandré Casagrande Figueiredo, James F Markworth, David Cameron-Smith
The mammalian target of rapamycin (mTOR) complex exerts a pivotal role in protein anabolism and cell growth. Despite its importance, few studies adequately address the complexity of phosphorylation of the mTOR protein itself to enable conclusions to be drawn on the extent of kinase activation following this event. In particular, a large number of studies in the skeletal muscle biology field have measured Serine 2448 (Ser2448) phosphorylation as a proxy of mTOR kinase activity. However, the evidence to be described is that Ser2448 is not a measure of mTOR kinase activity nor is a target of AKT activity and instead has inhibitory effects on the kinase that is targeted by the downstream effector p70S6K in a negative feedback loop mechanism, which is evident when revisiting muscle research studies...
February 20, 2017: Cellular and Molecular Life Sciences: CMLS
https://www.readbyqxmd.com/read/28219696/irisin-in-goldfish-carassius-auratus-effects-of-irisin-injections-on-feeding-behavior-and-expression-of-appetite-regulators-uncoupling-proteins-and-lipoprotein-lipase-and-fasting-induced-changes-in-fndc5-expression
#9
Zahndra Diann Butt, Jessica Dalton Hackett, Hélène Volkoff
Irisin is a peptide cleaved from the fibronectin type III domain containing protein 5 (FNDC5) gene that is secreted predominantly by muscle cells but also by other tissues including brain and intestine. In mammals, irisin has been shown to have thermogenic actions via the modulation of uncoupling proteins (UCPs) and to affect feeding and energy homeostasis via actions in brain, adipose tissue, liver, muscle and gastrointestinal tract. To examine the role of irisin on feeding and metabolism in fish, the effects of peripheral (intraperitoneal) injections of irisin on feeding behavior, glucose levels and the mRNA expressions of appetite regulators (cocaine and amphetamine regulated transcript CART, agouti related protein AgRP, orexin), UCPs and lipoprotein lipase LPL and brain factors (brain-derived neurotrophic factor , BDNF and tyrosine hydroxylase TH) were assessed in brain, white muscle and intestine...
February 17, 2017: Peptides
https://www.readbyqxmd.com/read/28219397/immunohistochemistry-of-sarcolemmal-membrane-associated-proteins-in-formalin-fixed-and-paraffin-embedded-skeletal-muscle-tissue-a-promising-tool-for-the-diagnostic-evaluation-of-common-muscular-dystrophies
#10
Chinnawut Suriyonplengsaeng, Charungthai Dejthevaporn, Chaiyos Khongkhatithum, Suda Sanpapant, Nattha Tubthong, Koset Pinpradap, Nippa Srinark, Jariya Waisayarat
BACKGROUND: The analysis of fresh frozen muscle specimens is standard following routine muscle biopsy, but this service is not widely available in countries with limited medical facilities, such as Thailand. Nevertheless, immunohistochemistry (IHC) analysis is essential for the diagnosis of patients with a strong clinical suspicion of muscular dystrophy, in the absence of mutations detected by molecular genetics. As the successful labelling of sarcolemmal membrane-associated proteins in formalin-fixed and paraffin-embedded (FFPE) muscle sections using IHC staining has rarely been described, this study aimed to develop a reproducible IHC method for such an analysis...
February 20, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28219134/-role-of-nuclear-factor-of-activated-t-cells-cytoplasmic-1-on-vascular-calcification-in-rats-with-chronic-renal-failure
#11
J X Zhang, J S Xu, Y Y Han, Y L Bai, L W Cui, H R Zhang, S L Zhang
Objective: To explore the role of nuclear factor of activated T-cells cytoplasmic 1 (NFATc1) on vascular calcification in chronic renal failure rats. Methods: Nineteen male Sprague-Dawley (SD) rats were randomly divided into three groups: sham-operated group (n=6), 5/6 Nephrectomy (Nx) group (n=6), 5/6 Nx+ calcitriol group (n=7). Vascular calcification was determined by von Kossa staining and orthocresolphthalein complexone (OCPC) method. Protein expressions of NFATc1 and runt-related transcription factor 2 (Runx2) in aortas were measured by immunohistochemistry...
February 14, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28219131/-recombinant-human-bone-morphogenetic-protein-7-inhibits-the-proliferation-and-extracellular-matrix-secretion-of-fibroblast-of-urethral-scars
#12
Y Wu, L M Ma, B Cai, H L Li, Q W Xing
Objective: To study the effect and its mechanism of recombinant human bone morphogenetic protein 7 (rhBMP-7) on urethral keloid fibroblast. Methods: Urethral fibroblasts were extracted and cultured, and the fourth-generation fibroblasts were used for experiment. Urethral fibroblasts were treated with rhBMP-7. Proliferation rate was detected by CCK-8 method. RT-PCR was applied to test changes of mRNA in α smooth muscle actin (α-SMA) and type Ⅰ collagen protein (COL-1) of each group. Western blot was used to measure α-SMA protein expression and the effect of rhBMP-7 on signaling pathway of urethral keloid fibroblasts...
February 14, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28218659/effects-of-remote-ischemic-preconditioning-on-heme-oxygenase-1-expression-and-cutaneous-wound-repair
#13
Niels A J Cremers, Kimberley E Wever, Ronald J Wong, René E M van Rheden, Eline A Vermeij, Gooitzen M van Dam, Carine E Carels, Ditte M S Lundvig, Frank A D T G Wagener
Skin wounds may lead to scar formation and impaired functionality. Remote ischemic preconditioning (RIPC) can induce the anti-inflammatory enzyme heme oxygenase-1 (HO-1) and protect against tissue injury. We aim to improve cutaneous wound repair by RIPC treatment via induction of HO-1. RIPC was applied to HO-1-luc transgenic mice and HO-1 promoter activity and mRNA expression in skin and several other organs were determined in real-time. In parallel, RIPC was applied directly or 24h prior to excisional wounding in mice to investigate the early and late protective effects of RIPC on cutaneous wound repair, respectively...
February 17, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28218619/muscular-dystrophy-meets-protein-biochemistry-the-mother-of-invention
#14
Steven D Funk, Jeffrey H Miner
Muscular dystrophies result from a defect in the linkage between the muscle fiber cytoskeleton and the basement membrane (BM). Congenital muscular dystrophy type MDC1A is caused by mutations in laminin α2 that either reduce its expression or impair its ability to polymerize within the muscle fiber BM. Defects in this BM lead to muscle fiber damage from the force of contraction. In this issue of the JCI, McKee and colleagues use a laminin polymerization-competent, designer chimeric BM protein in vivo to restore function of a polymerization-defective laminin, leading to normalized muscle structure and strength in a mouse model of MDC1A...
February 20, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28218617/chimeric-protein-repair-of-laminin-polymerization-ameliorates-muscular-dystrophy-phenotype
#15
Karen K McKee, Stephanie C Crosson, Sarina Meinen, Judith R Reinhard, Markus A Rüegg, Peter D Yurchenco
Mutations in laminin α2-subunit (Lmα2, encoded by LAMA2) are linked to approximately 30% of congenital muscular dystrophy cases. Mice with a homozygous mutation in Lama2 (dy2J mice) express a nonpolymerizing form of laminin-211 (Lm211) and are a model for ambulatory-type Lmα2-deficient muscular dystrophy. Here, we developed transgenic dy2J mice with muscle-specific expression of αLNNd, a laminin/nidogen chimeric protein that provides a missing polymerization domain. Muscle-specific expression of αLNNd in dy2J mice resulted in strong amelioration of the dystrophic phenotype, manifested by the prevention of fibrosis and restoration of forelimb grip strength...
February 20, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28218490/comparative-effects-of-energy-restriction-and-resveratrol-intake-on-glycemic-control-improvement
#16
I Milton-Laskibar, L Aguirre, M T Macarulla, U Etxeberria, F I Milagro, J A Martínez, J Contreras, M P Portillo
Resveratrol (RSV) has been proposed as an energy restriction mimetic. This study aimed to compare the effects of RSV and energy restriction on insulin resistance induced by an obesogenic diet. Any additive effect of both treatments was also analyzed. Rats were fed a high-fat high-sucrose diet for 6 weeks. They were then distributed in four experimental groups which were either fed a standard control diet (C), or treated with RSV (30 mg/kg/d), or submitted to energy restriction (R, 15%), or treated with RSV and submitted to energy restriction (RR)...
February 20, 2017: BioFactors
https://www.readbyqxmd.com/read/28217832/gestational-protein-restriction-study-of-the-probable-effects-on-cardiac-muscle-structure-and-function-in-adult-rats
#17
Mona G Amer, Nader M Mohamed, Aly A M Shaalan
Intrauterine growth restriction (IUGR) has been linked to heart disease in adulthood. This study aimed to examine the effect of gestational protein restriction during fetal and early postnatal life on the cardiac muscle structure and function in adult offspring. Pregnant female rats were randomly divided into two dietary groups: normal-protein diet (NP) and low-protein diet (LP). Fifteen male offspring from each group were included in the study. Offspring body weights were recorded at birth and monthly from weaning until 24 weeks of age while systolic blood pressure was measured weekly...
February 20, 2017: Histology and Histopathology
https://www.readbyqxmd.com/read/28217807/-role-of-trpc6-in-pulmonary-artery-smooth-muscle-cells-proliferation-and-apoptosis-under-hypoxia-and-hypercapnia
#18
Xu-Guang Jia, Meng-Xiao Zheng, Jing-Jing Zhang, Cong-Cong Zhang, Mei-Ping Zhao, Yi-Ming Wu, Xi-Wen Chen, Wan-Tie Wang
The present study was to investigate the role of TRPC6 in pulmonary artery smooth muscle cells (PASMCs) proliferation and apoptosis under hypoxia and hypercapnia. PASMCs were isolated from chloral hydrate-anesthetized male Sprague-Dawley (SD) rats. Cellular purity was assessed by immunofluorescence staining for smooth muscle α-actin under fluorescence microscopy. Passage 4-6 PASMCs were starved for 24 h in serum-free DMEM and divided into 5 groups randomly: normoxia, hypoxia and hypercapnia, DMSO, TRPC6 inhibitor SKF-96365 and TRPC6 activator OAG groups...
February 25, 2017: Sheng Li Xue Bao: [Acta Physiologica Sinica]
https://www.readbyqxmd.com/read/28217217/the-effect-of-air-tourniquet-on-interleukin-6-levels-in-total-knee-arthroplasty
#19
Kenji Tsunoda, Motoki Sonohata, Hajime Kugisaki, Shinsuke Someya, Hidefumi Honke, Mitsunori Komine, Masataka Izumi, Shuya Ide, Masaaki Mawatari
BACKGROUND: Air tourniquet-induced skeletal muscle injury increases the concentrations of some cytokines such as interleukin-6 (IL-6) in plasma. However, the effect of an air tourniquet on the IL-6 concentrations after total knee arthroplasty (TKA) is unclear. We therefore investigated the impact of tourniquet-induced ischemia and reperfusion injury in TKA using the IL-6 level as an index. METHODS: Ten patients with primary knee osteoarthrosis who underwent unilateral TKA without an air tourniquet were recruited (Non-tourniquet group)...
2017: Open Orthopaedics Journal
https://www.readbyqxmd.com/read/28217183/affection-of-the-respiratory-muscles-in-combined-complex-i-and-iv-deficiency
#20
Josef Finsterer, Helmut Rauschka, Liane Segal, Gabor G Kovacs, Boris Rolinski
OBJECTIVES: Combined complex I+IV deficiency has rarely been reported to manifest with the involvement of the respiratory muscles. CASE REPORT: A 45y male was admitted for hypercapnia due to muscular respiratory insufficiency. He required intubation and mechanical ventilation. He had a previous history of ophthalmoparesis since age 6y, ptosis since age 23y, and anterocollis since at least age 40y. Muscle biopsy from the right deltoid muscle at age 41y was indicative of mitochondrial myopathy...
2017: Open Neurology Journal
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