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https://www.readbyqxmd.com/read/27921261/attempting-to-compensate-for-reduced-neuronal-nitric-oxide-synthase-protein-with-nitrate-supplementation-cannot-overcome-metabolic-dysfunction-but-rather-has-detrimental-effects-in-dystrophin-deficient-mdx-muscle
#1
Cara A Timpani, Adam J Trewin, Vanesa Stojanovska, Ainsley Robinson, Craig A Goodman, Kulmira Nurgali, Andrew C Betik, Nigel Stepto, Alan Hayes, Glenn K McConell, Emma Rybalka
Duchenne muscular dystrophy arises from the loss of dystrophin and is characterized by calcium dysregulation, muscular atrophy, and metabolic dysfunction. The secondary reduction of neuronal nitric oxide synthase (nNOS) from the sarcolemma reduces NO production and bioavailability. As NO modulates glucose uptake, metabolism, and mitochondrial bioenergetics, we investigated whether an 8-week nitrate supplementation regimen could overcome metabolic dysfunction in the mdx mouse. Dystrophin-positive control (C57BL/10) and dystrophin-deficient mdx mice were supplemented with sodium nitrate (85 mg/l) in drinking water...
December 5, 2016: Neurotherapeutics: the Journal of the American Society for Experimental NeuroTherapeutics
https://www.readbyqxmd.com/read/27907044/coenzyme-q10-status-as-a-determinant-of-muscular-strength-in-two-independent-cohorts
#2
Alexandra Fischer, Simone Onur, Petra Niklowitz, Thomas Menke, Matthias Laudes, Gerald Rimbach, Frank Döring
Aging is associated with sarcopenia, which is a loss of skeletal muscle mass and function. Coenzyme Q10 (CoQ10) is involved in several important functions that are related to bioenergetics and protection against oxidative damage; however, the role of CoQ10 as a determinant of muscular strength is not well documented. The aim of the present study was to evaluate the determinants of muscular strength by examining hand grip force in relation to CoQ10 status, gender, age and body mass index (BMI) in two independent cohorts (n = 334, n = 967)...
2016: PloS One
https://www.readbyqxmd.com/read/27906078/four-week-rapamycin-treatment-improves-muscular-dystrophy-in-a-fukutin-deficient-mouse-model-of-dystroglycanopathy
#3
Steven J Foltz, Junna Luan, Jarrod A Call, Ankit Patel, Kristen B Peissig, Marisa J Fortunato, Aaron M Beedle
BACKGROUND: Secondary dystroglycanopathies are a subset of muscular dystrophy caused by abnormal glycosylation of α-dystroglycan (αDG). Loss of αDG functional glycosylation prevents it from binding to laminin and other extracellular matrix receptors, causing muscular dystrophy. Mutations in a number of genes, including FKTN (fukutin), disrupt αDG glycosylation. METHODS: We analyzed conditional Fktn knockout (Fktn KO) muscle for levels of mTOR signaling pathway proteins by Western blot...
June 2, 2016: Skeletal Muscle
https://www.readbyqxmd.com/read/27904496/adipose-derived-stem-cells-enhance-myogenic-differentiation-in-the-mdx-mouse-model-of-muscular-dystrophy-via-paracrine-signaling
#4
Ji-Qing Cao, Ying-Yin Liang, Ya-Qin Li, Hui-Li Zhang, Yu-Ling Zhu, Jia Geng, Li-Qing Yang, Shan-Wei Feng, Juan Yang, Jie Kong, Cheng Zhang
Adipose-derived stem cells have been shown to promote peripheral nerve regeneration through the paracrine secretion of neurotrophic factors. However, it is unclear whether these cells can promote myogenic differentiation in muscular dystrophy. Adipose-derived stem cells (6 × 10(6)) were injected into the gastrocnemius muscle of mdx mice at various sites. Dystrophin expression was found in the muscle fibers. Phosphorylation levels of Akt, mammalian target of rapamycin (mTOR), eIF-4E binding protein 1 and S6 kinase 1 were increased, and the Akt/mTOR pathway was activated...
October 2016: Neural Regeneration Research
https://www.readbyqxmd.com/read/27897115/cysteinet-dysregulation-in-muscular-dystrophies-a-pathogenic-network-susceptible-to-therapy
#5
Marcos Arturo Martínez-Banaclocha
BACKGROUND: Muscular dystrophies are inherited disorders characterized by progressive skeletal muscle degeneration without curative therapy. The specific defective protein in each type of muscular dystrophy has been associated with different deleterious factors that contribute to the progression of the disease. Among these factors, the impairment of calcium homeostasis, the ubiquitin-proteasome dysfunction, and the oxidative damage of cellular macromolecules seem to be of central importance...
November 29, 2016: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/27894297/pulmonary-hypertension-and-vascular-remodeling-in-mice-exposed-to-crystalline-silica
#6
Igor N Zelko, Jianxin Zhu, Jeffrey D Ritzenthaler, Jesse Roman
BACKGROUND: Occupational and environmental exposure to crystalline silica may lead to the development of silicosis, which is characterized by inflammation and progressive fibrosis. A substantial number of patients diagnosed with silicosis develop pulmonary hypertension. Pulmonary hypertension associated with silicosis and with related restrictive lung diseases significantly reduces survival in affected subjects. An animal model of silicosis has been described previously however, the magnitude of vascular remodeling and hemodynamic effects of inhaled silica are largely unknown...
November 28, 2016: Respiratory Research
https://www.readbyqxmd.com/read/27890884/correspondence-and-challenges-as-neurologists-to-kumamoto-earthquakes-in-2016
#7
Makoto Nakajima, Nozomi Nakane, Kotaro Takamatsu, Satoshi Yamashita, Shunya Nakane, Taro Yamashita, Yukio Ando
Kumamoto Earthquakes in 2016 severely affected medical circumstances and condition of each patient with neuro-muscular diseases, in addition to having destroyed life circumstances of local residence. Number of neuro-muscular disease patients admitted to the Department of Neurology, Kumamoto University, the only university hospital in the prefecture, increased approximately twice compared to usual years. Most of the related facilities were able to admit emergency patients with neuro-muscular diseases although the hospital buildings were damaged in various degrees...
November 25, 2016: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/27887661/biochemical-and-proteomic-analyses-of-the-physiological-response-induced-by-individual-housing-in-gilts-provide-new-potential-stress-markers
#8
Anna Marco-Ramell, Laura Arroyo, Raquel Peña, Raquel Pato, Yolanda Saco, Lorenzo Fraile, Emøke Bendixen, Anna Bassols
BACKGROUND: The objective assessment of animal stress and welfare requires proper laboratory biomarkers. In this work, we have analyzed the changes in serum composition in gilts after switching their housing, from pen to individual stalls, which is generally accepted to cause animal discomfort. RESULTS: Blood and saliva samples were collected a day before and up to four days after changing the housing system. Biochemical analyses showed adaptive changes in lipid and protein metabolism after the housing switch, whereas cortisol and muscular markers showed a large variability between animals...
November 25, 2016: BMC Veterinary Research
https://www.readbyqxmd.com/read/27885178/-changes-in-cytosolic-ca-2-dynamics-associated-with-muscular-dystrophy
#9
Jun Tanihata, Shin'ichi Takeda
Duchenne muscular dystrophy(DMD)is X-linked genetic disorder caused by a lack of the membrane-associated protein dystrophin. DMD is characterized by progressive muscle wasting secondary to repeated muscle damage and inadequate repair. The mechanisms underlying the functional impairments in dystrophic muscle have not yet been fully determined. However, several recent studies indicate that elevated intracellular Ca(2+)homeostasis is a cause or facilitator of the development of muscle weakness in muscular dystrophy...
2016: Clinical Calcium
https://www.readbyqxmd.com/read/27881060/mtdna-damage-associated-with-mitochondrial-cytopathies
#10
Anastasia I Ryzhkova, Alexandra A Melnichenko, Zukhra B Khasanova, Andrey V Zhelankin, Konstantin Y Mitrofanov, Anton Yu Postnov, Alexander N Orekhov, Igor A Sobenin
Mitochondrial cytopathies are a group of diseases which occur in case of disturbance of the functional activity of mitochondria. Mitochondrial diseases are a heterogeneous group of multiple organ pathologies, mainly affecting the nervous and muscular systems. The causes of mitochondrial cytopathies may be nuclear genome mutations occurring in genes coding mitochondrial proteins and mitochondrial genome mutations which occur sporadically or maternally inherited. At the present time many factors leading to the occurrence of mutations are known...
November 23, 2016: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/27869890/-anmco-sic-sici-gise-sicch-consensus-document-risk-stratification-in-elderly-patients-undergoing-cardiac-surgery-and-transcatheter-aortic-valve-implantation
#11
Giovanni Pulignano, Michele Massimo Gulizia, Samuele Baldasseroni, Francesco Bedogni, Giovanni Cioffi, Ciro Indolfi, Francesco Romeo, Adriano Murrone, Francesco Musumeci, Alessandro Parolari, Leonardo Patanè, Paolo Giuseppe Pino, Annalisa Mongiardo, Carmen Spaccarotella, Roberto Di Bartolomeo, Giuseppe Musumeci
Aortic stenosis is one the most frequent valvular diseases in developed countries, and its impact on public healthcare resources and assistance is increasing. A substantial proportion of elderly patients with severe aortic stenosis is frequently not eligible for surgery because of advanced age, frailty and multiple comorbidities. Transcatheter aortic valve implantation (TAVI) enables the treatment of very elderly patients at high or prohibitive surgical risk considered ineligible for surgery and with an acceptable life expectancy...
September 2016: Giornale Italiano di Cardiologia
https://www.readbyqxmd.com/read/27859827/elevated-phosphodiester-and-t2-levels-can-be-measured-in-the-absence-of-fat-infiltration-in-duchenne-muscular-dystrophy-patients
#12
M T Hooijmans, E H Niks, J Burakiewicz, J J G M Verschuuren, A G Webb, H E Kan
Quantitative MRI and MRS are increasingly important as non-invasive outcome measures in therapy development for Duchenne muscular dystrophy (DMD). Many studies have focussed on individual measures such as fat fraction and metabolite levels in relation to age and functionality, but much less attention has been given to how these indices relate to each other. Here, we assessed spatially resolved metabolic changes in leg muscles of DMD patients, and classified muscles according to the degree of fat replacement compared with healthy controls...
November 17, 2016: NMR in Biomedicine
https://www.readbyqxmd.com/read/27855487/transfer-of-therapeutic-genes-into-fetal-rhesus-monkeys-using-recombinant-adeno-associated-type-i-viral-vectors
#13
Thomas J Conlon, Cathryn S Mah, Christina A R Pacak, Mary B Rucker Henninger, Kirsten E Erger, Marda L Jorgensen, Charles C Lee, Alice F Tarantal, Barry J Byrne
Neuromuscular disorders such as Pompe disease (glycogen storage disease, type II), result in early and potentially irreversible cellular damage with a very limited opportunity for intervention in the newborn period. Pompe disease is due to deficiency in acid α-glucosidase (GAA) leading to lysosomal accumulation of glycogen in all cell types, abnormal myofibrillogenesis, respiratory insufficiency, neurological deficits, and reduced contractile function in striated muscle. Previous studies have shown that fetal delivery of recombinant adeno-associated virus (rAAV) encoding GAA to the peritoneal cavity of Gaa-/- mice resulted in high-level transduction of the diaphragm...
November 17, 2016: Human Gene Therapy. Clinical Development
https://www.readbyqxmd.com/read/27849484/the-benefits-of-high-intensity-functional-training-fitness-programs-for-military-personnel
#14
Christopher K Haddock, Walker S C Poston, Katie M Heinrich, Sara A Jahnke, Nattinee Jitnarin
INTRODUCTION: High intensity functional training (HIFT) programs are designed to address multiple fitness domains, potentially providing improved physical and mental readiness in a changing operational environment. Programs consistent with HIFT principals such as CrossFit, SEALFIT and the US Marine Corps' High Intensity Tactical Training (HITT) are increasingly popular among military personnel. The goal of HIFT programs is to produce high levels of cardiorespiratory fitness, endurance and strength that exceed those achieved by following current physical activity recommendations...
November 2016: Military Medicine
https://www.readbyqxmd.com/read/27843426/successful-endoscopic-submucosal-dissection-of-a-large-terminal-ileal-lipoma
#15
Hisatsugu Noda, Naotaka Ogasawara, Yasuhiro Tamura, Yoshihiro Kondo, Shinya Izawa, Masahide Ebi, Yasushi Funaki, Makoto Sasaki, Kunio Kasugai
A 78-year-old woman who had recurrent right lower abdominal pain for about 1 year underwent computed tomography (CT) because of a follow-up observation 1 year after right breast cancer surgery. CT revealed a tumor in the colon. The patient was referred to our hospital for detailed examinations. An abdominal CT showed a low-density tumor of approximately 30 mm in the ascending colon, and the CT density inside the tumor was same as that of fatty tissues. A subsequent colonoscopy showed a submucosal tumor (SMT) in the proximal ascending colon developing from the terminal ileum...
September 2016: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/27836895/lifelong-quercetin-enrichment-and-cardioprotection-in-mdx-utrn-ice
#16
Christopher Ballmann, Thomas Denney, Ronald J Beyers, Tiffany S Quindry, Matthew Romero, Rajesh H Amin, Joshua T Selsby, John C Quindry
Duchenne Muscular Dystrophy (DMD) is associated with progressive cardiac pathology, however, the SIRT1/PGC1-α activator quercetin may cardioprotect dystrophic hearts. We tested the extent to which long term 0.2% dietary quercetin enrichment attenuates dystrophic cardiopathology in Mdx/Utrn(+/-) mice. At 2 months, Mdx/Utrn(+/-) mice were fed quercetin enriched (Mdx/Utrn(+/-)-Q) or control diet (Mdx/Utrn(+/-)) for 8 months. Control C57BL/10 (C57) animals were fed a control diet for 10 months. Cardiac function was quantified by MRI at 2 and 10 months...
November 11, 2016: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/27830982/excretion-and-toxicity-evaluation-of-131-i-sennoside-a-as-a-necrosis-avid-agent
#17
Zhiqi Yin, Lidan Sun, Qiaomei Jin, Shaoli Song, Yuanbo Feng, Hong Liao, Yicheng Ni, Jian Zhang, Wei Liu
1. Sennoside A (SA) is a newly identified necrosis avid agent that shows capability for imaging diagnosis and tumor therapy. As a water-soluble compound, (131)I-Sennoside A ((131)I-SA) might be excreted mainly through the kidneys with possibility of nephrotoxicity. 2. To further verify excretion pathway and examine nephrotoxicity of (131)I-SA, excretion, and nephrotoxicity were appraised. The pharmacokinetics, hepatotoxicity and hematotoxicity of (131)I-SA were also evaluated to accelerate its possible clinical translation...
November 10, 2016: Xenobiotica; the Fate of Foreign Compounds in Biological Systems
https://www.readbyqxmd.com/read/27825052/identification-of-potential-compensatory-muscle-strategies-in-a-breast-cancer-survivor-population-a-combined-computational-and-experimental-approach
#18
Jaclyn N Chopp-Hurley, Rebecca L Brookham, Clark R Dickerson
BACKGROUND: Biomechanical models are often used to estimate the muscular demands of various activities. However, specific muscle dysfunctions typical of unique clinical populations are rarely considered. Due to iatrogenic tissue damage, pectoralis major capability is markedly reduced in breast cancer population survivors, which could influence arm internal and external rotation muscular strategies. METHODS: Accordingly, an optimization-based muscle force prediction model was systematically modified to emulate breast cancer population survivors through adjusting pectoralis capability and enforcing an empirical muscular co-activation relationship...
October 29, 2016: Clinical Biomechanics
https://www.readbyqxmd.com/read/27822449/similar-efficacy-from-specific-and-non-specific-mineralocorticoid-receptor-antagonist-treatment-of-muscular-dystrophy-mice
#19
Jeovanna Lowe, Kyle T Floyd, Neha Rastogi, Eric J Schultz, Jessica A Chadwick, Sarah A Swager, Jonathan G Zins, Feni K Kadakia, Suzanne Smart, Elise P Gomez-Sanchez, Celso E Gomez-Sanchez, Subha V Raman, Paul M L Janssen, Jill A Rafael-Fortney
BACKGROUND: Combined treatment with an angiotensin-converting enzyme inhibitor and a mineralocorticoid receptor (MR) antagonist improved cardiac and skeletal muscle function and pathology in a mouse model of Duchenne muscular dystrophy. MR is present in limb and respiratory skeletal muscles and functions as a steroid hormone receptor. OBJECTIVE: The goals of the current study were to compare the efficacy of the specific MR antagonist eplerenone with the non-specific MR antagonist spironolactone, both in combination with the angiotensin-converting enzyme inhibitor lisinopril...
2016: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/27807335/-clinical-features-of-a-genetically-identified-spinal-and-%C3%A2-bulbar-muscular-atrophy-pedigree
#20
Zhe Wang, Qihua Chen, Qiuxiang Li, Fangfang Bi
Spinal and bulbar muscular atrophy (SBMA) is a rare X-linked motor neuron disease with significant phenotypic viability. Here, we present a genetically identified SBMA family without bulbar paralysis or androgen insensitivity. All four male patients presented with progressive lower motor neuron paralysis in all limbs, with distal extremities more dominant. None of them had bulbar palsy or androgen insensitivity. A consistently mild elevated blood creatine phosphokinase (CPK) levels were detected in all patients and the EMG showed a chronic neurogenic damage...
October 28, 2016: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
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