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Muscle Disease

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https://www.readbyqxmd.com/read/28806761/transcriptome-analysis-of-pdgfr%C3%AE-cells-identifies-t-type-ca2-channel-cacna1g-as-a-new-pathological-marker-for-pdgfr%C3%AE-cell-hyperplasia
#1
Se Eun Ha, Moon Young Lee, Masaaki Kurahashi, Lai Wei, Brian G Jorgensen, Chanjae Park, Paul J Park, Doug Redelman, Kent C Sasse, Laren S Becker, Kenton M Sanders, Seungil Ro
Platelet-derived growth factor receptor alpha (PDGFRα)+ cells are distributed into distinct morphological groups within the serosal, muscular, and submucosal layers as well as the myenteric and deep muscular plexi. PDGFRα+ cells directly interact with interstitial cells of Cajal (ICC) and smooth muscle cells (SMC) in gastrointestinal smooth muscle tissue. These three cell types, SMC, ICC, and PDGFRα+ cells (SIP cells), form an electrical syncytium, which dynamically regulates gastrointestinal motility. We have previously reported the transcriptomes of SMC and ICC...
2017: PloS One
https://www.readbyqxmd.com/read/28806178/vitamin-d-and-osteosarcopenia-an-update-from-epidemiological-studies
#2
Olivier Bruyère, Etienne Cavalier, Jean-Yves Reginster
PURPOSE OF REVIEW: The review summarizes recent epidemiological studies that examined the relationship between osteoporosis and sarcopenia to assess the impact of vitamin D status or supplementation on health outcomes related to these two medical conditions. RECENT FINDINGS: Osteoporosis and sarcopenia are major public health problems, but whether these two diseases should be considered alone or combined into a single condition is not clear. No consensual definition of osteosarcopenia is largely accepted...
August 12, 2017: Current Opinion in Clinical Nutrition and Metabolic Care
https://www.readbyqxmd.com/read/28806139/mask-mitigates-mapt-and-fus-induced-degeneration-by-enhancing-autophagy-through-lysosomal-acidification
#3
Mingwei Zhu, Sheng Zhang, Xiaolin Tian, Chunlai Wu
Accumulation of intracellular misfolded or damaged proteins is associated with both normal aging and late-onset degenerative diseases. Two cellular clearance mechanisms, the ubiquitin-proteasome system (UPS) and the macroautophagy/autophagy-lysosomal pathway, work in concert to degrade harmful protein aggregates and maintain protein homeostasis. Here we show that Mask, an Ankyrin-repeat and KH-domain containing protein, plays a key role in promoting autophagy flux and mitigating degeneration caused by protein aggregation or impaired UPS function...
August 14, 2017: Autophagy
https://www.readbyqxmd.com/read/28805762/-a-study-of-combination-treatment-with-nacom-levodopa-carbodope-and-citicoline-in-the-model-of-parkinson-disease-in-rats
#4
V A Kashkin, E V Shekunova, M N Makarova, V G Makarov
AIM: To evaluate the efficacy of the combined use of citicoline (neipilept) and levodopa/carbidopa (nakom) in the rotenone model of Parkinson's disease in rats. MATERIAL AND METHODS: Rotenone was administrated during 14 days in dose 2 mg/kg/day subcutaneously. The duration of treatment was 7 days, intragastrically. Alteration of locomotor behavior components, muscular rigidity in resistance to passive flexion in the ankle joint and signs of extrapyramidal disorders were assessed...
2017: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
https://www.readbyqxmd.com/read/28805065/neurohormonal-modulation-for-treatment-of-cardiac-involvement-in-dystrophinopathies-and-mitochondrial-disease
#5
Alberto Aimo, Alberto Giannoni, Vincenzo Castiglione, Michelangelo Mancuso, Gabriele Siciliano, Massimo F Piepoli, Claudio Passino, Michele Emdin
Mutations in either the nuclear or the mitochondrial genome can lead to structural and functional changes within the skeletal muscles. These genetic skeletal myopathies are rare, although not infrequent when their cumulative incidence is considered. Dystrophinopathies (Duchenne and Becker muscular dystrophies) and mitochondrial disease are some of the most frequent clinical entities, and those developing heart failure more frequently. Neurohormonal antagonism represents the cornerstone of heart failure management, even though its role in the prevention and treatment of heart failure in patients with dystrophinopathies or mitochondrial disorders remains undefined...
January 1, 2017: European Journal of Preventive Cardiology
https://www.readbyqxmd.com/read/28805016/microvascular-abnormalities-assessed-by-nailfold-capillaroscopy-in-juvenile-dermatomyositis-after-medium-to-long-term-follow-up
#6
Zoltan Barth, Birgit N Witczak, Berit Flatø, Akos Koller, Ivar Sjaastad, Helga Sanner
OBJECTIVE: In juvenile dermatomyositis (JDM), microvascular abnormalities measured by nailfold capillaroscopy (NFC) are common early in disease course. We aimed to compare the presence of NFC abnormalities in patients with medium- to longstanding JDM with that of controls, and to explore associations between NFC abnormalities and disease activity and other disease characteristics. METHODS: Fifty-eight JDM patients clinically examined median 16.8 (2-38) years after disease onset were compared with matched controls...
August 13, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/28804634/muscle-mri-findings-in-a-one-year-old-girl-with-merosin-deficient-congenital-muscular-dystrophy-type-1a-due-to-lama2-mutation-a-case-report
#7
Yingyin Liang, Guidian Li, Songlin Chen, Rongxing He, Xiangxue Zhou, Yingming Chen, Xue Xu, Ronglan Zhu, Cheng Zhang
The objective of the present study was to characterize the muscle magnetic resonance imaging (MRI) features of a 1-year-old girl with merosin-deficient congenital muscular dystrophy type 1A (MDC1A). Beginning as an infant, this patient exhibited severe hypotonia and proximal weakness, as well as delays in developmental milestones. Her serum creatine kinase levels at 3 months, 8 months and 1 year were 2,959, 1,621 and 1,659 U/l, respectively. Brain MRI indicated symmetric, mild T1WI low, mild T2WI and FLAIR high radial patterns in the white matter of the Cornu posterius of the ventricular lateral...
August 2017: Biomedical Reports
https://www.readbyqxmd.com/read/28804461/the-effect-of-resistance-exercise-on-inflammatory-and-myogenic-markers-in-patients-with-chronic-kidney-disease
#8
Emma L Watson, Joao L Viana, David Wimbury, Naomi Martin, Neil J Greening, Jonathan Barratt, Alice C Smith
Background: Muscle wasting is a common complication of Chronic Kidney Disease (CKD) and is clinically important given its strong association with morbidity and mortality in many other chronic conditions. Exercise provides physiological benefits for CKD patients, however the molecular response to exercise remains to be fully determined. We investigated the inflammatory and molecular response to resistance exercise before and after training in these patients. Methods: This is a secondary analysis of a randomized trial that investigated the effect of 8 week progressive resistance training on muscle mass and strength compared to non-exercising controls...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28803986/ubiquitin-c-terminal-hydrolase-l1-regulates-myoblast-proliferation-and-differentiation
#9
Hongbo Gao, Sigurd Hartnett, Yifan Li
Skeletal muscles are dynamic tissues that possess regenerative abilities, which require multiple processes and regulatory factors. Ubiquitin C-Terminal Hydrolase L1 (UCHL1), which is primarily expressed in neuronal tissues, was upregulated in skeletal muscles in disease conditions but its functional role in skeletal muscles is unknown. Using mouse myoblast cells C2C12 as an in vitro model, this study reported that UCHL1 elicits different regulation in myoblast cell proliferation and differentiation. We first observed that UCHL1 protein level was continuously declined during cell differentiation...
August 10, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28803741/the-cardiovascular-toxicity-of-triadimefon-in-early-life-stage-of-zebrafish-and-potential-implications-to-human-health
#10
Hong-Cui Liu, Tian-Yi Chu, Li-Li Chen, Wen-Jun Gui, Guo-Nian Zhu
The health risk of triadimefon (TF) to cardiovascular system of human is still unclear, especially to pesticide suicides population, occupational population (farmers, retailers and pharmaceutical workers), and special population (young children and infants, pregnant women, older people, and those with compromised immune systems) who are at a greater risk. Therefore, firstly we explored the toxic effects and possible mechanism of cardiovascular toxicity induced by TF using zebrafish model. Zebrafish at stage of 48 h post fertilization (hpf) exposed to TF for 24 h exhibited morphological malformations which were further confirmed by histopathologic examination, including pericardial edema, circulation abnormalities, serious venous thrombosis and increased distance between the sinus venosus (SV) and bulbus arteriosus (BA) regions of the heart...
August 10, 2017: Environmental Pollution
https://www.readbyqxmd.com/read/28803683/immune-response-in-takayasu-arteritis
#11
Tristan Mirault, Henri Guillet, Emmanuel Messas
Takayasu arteritis (TAK) is a vasculitis of the large arteries. The arterial wall, target of the immune reaction, is composed of vascular dendritic cells, endothelial cells, vascular smooth muscle cells and fibroblasts, which engage in an interaction with T cells and macrophages to, ultimately, cause luminal stenosis or aneurysmal wall damage of the vessel. A multitude of cytokines have been identified to increase in case of TAK and to be linked to disease activity. A better understanding of the physiopathological pathways and mechanisms involved, might enable a more tailored therapeutic approach in TAK...
August 10, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28803422/salidroside-improved-cerebrovascular-vasodilation-in-streptozotocin-induced-diabetic-rats-through-restoring-the-function-of-bkca-channel-in-smooth-muscle-cells
#12
Yu-Guang Ma, Jun-Wei Wang, Yin-Bin Zhang, Bao-Feng Wang, Zhi-Jun Dai, Man-Jiang Xie, Hua-Feng Kang
Vessel disease is a kind of severe complication in diabetic patients. However, few pharmacologic agents can directly recover diabetic vascular function. Salidroside (SAL), a major ingredient from Rhodiola rosea, has been found to have an obvious hypoglycemic effect and a beneficial protection on vascular function in diabetes. However, whether SAL is a suitable treatment for diabetes has not so far been evaluated and the underlying mechanisms remain unknown. The present work aims to (1) investigate the potential effects of SAL on cerebrovascular relaxation in streptozotocin-induced diabetic rats or when exposed to acute hyperglycemia condition and (2) examine whether function of the BKCa channel is involved in SAL treatment for diabetic vascular relaxation...
August 12, 2017: Cell and Tissue Research
https://www.readbyqxmd.com/read/28803350/comparison-of-mitral-competence-after-mitral-repair-with-papillary-muscle-approximation-versus-papillary-muscle-relocation-for-functional-mitral-regurgitation
#13
Koji Furukawa, Mitsuhiro Yano, Eisaku Nakamura, Masakazu Matsuyama, Masanori Nishimura, Katsuya Kawagoe, Kunihide Nakamura
The purpose of this study was to evaluate the surgical results of papillary muscle approximation (PMA) and papillary muscle relocation (PMR) for functional mitral regurgitation (FMR) and to compare the effects of both procedures on the change in mitral regurgitation (MR) and echocardiogram parameters associated with tethering. Eighteen patients with moderate-to-severe FMR (MR grade ≥2) who underwent PMA or PMR were retrospectively analyzed. Underlying diseases were ischemic cardiomyopathy, idiopathic dilated cardiomyopathy, and aortic valve disease for seven, six, and five patients, respectively...
August 12, 2017: Heart and Vessels
https://www.readbyqxmd.com/read/28802887/clinical-outcomes-of-patients-with-t1-nested-variant-of-urothelial-carcinoma-compared-to-pure-urothelial-carcinoma-of-the-bladder
#14
Abhijith D Mally, Amy L Tin, Justin K Lee, Prassannah Satasivam, Eugene K Cha, S Michele Donat, Harry W Herr, Bernard H Bochner, Daniel D Sjoberg, Guido Dalbagni
PURPOSE: Evaluate oncologic outcomes of patients with cT1 nested variant (NV) of urothelial carcinoma (UC) and compare with cases of pure UC of the bladder. MATERIALS AND METHODS: We retrospectively identified 30 patients with NV who, between 1997 and 2012, underwent transurethral resection with T1 tumor stage, followed by restaging transurethral resection within 3 months confirming non-muscle-invasive disease. Radical cystectomy within 3 months of restaging transurethral resection was considered "early" treatment...
July 14, 2017: Clinical Genitourinary Cancer
https://www.readbyqxmd.com/read/28802220/comparison-of-lower-limb-muscle-strength-between-diabetic-neuropathic-and-healthy-subjects-using-opensim
#15
Alessandra Scarton, Ilse Jonkers, Annamaria Guiotto, Fabiola Spolaor, Gabriella Guarneri, Angelo Avogaro, Claudio Cobelli, Zimi Sawacha
Diabetes neuropathy and vasculopathy are the two major complications of diabetes mellitus, leading to diabetic foot disease, of which the worst consequences are plantar ulcers and amputations. Motor impairments like joint stiffness and loss of balance are distinctive effects of diabetes and they have been extensively explored. However, while altered muscle function has been also assessed through experimentally measured surface electromyography, little is known about muscle forces. The objective of this study was to estimate muscle forces in subjects with diabetes and to use these data to identify differences with respect to a population of healthy subjects matched for age and BMI...
July 31, 2017: Gait & Posture
https://www.readbyqxmd.com/read/28801956/evidence-of-er-stress-and-upr-activation-in-patients-with-brody-disease-and-brody-syndrome
#16
V Guglielmi, N C Voermans, A Oosterhof, D Nowis, B G van Engelen, G Tomelleri, G Vattemi
Brody disease (BD, OMIM #601003) is an inherited skeletal muscle disease clinically characterized by exercise-induced impairment of muscle relaxation and stiffness due to the delay in the Ca(2+) re-uptake in the sarcoplasmic reticulum (SR)[1, 2]. The delayed muscle relaxation mainly affects legs, harms, hands and eyelids and usually improves after a few minutes rest [1-3]. Patients frequently report myalgia, painless or mildly painful cramps whereas recurrent rhabdomyolysis have been described in a few cases [2, 3]...
August 12, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28801444/radical-cystectomy-bladder-removal-against-intravesical-bcg-immunotherapy-for-high-risk-non-muscle-invasive-bladder-cancer-bravo-a-protocol-for-a-randomised-controlled-feasibility-study
#17
Jamie B Oughton, Heather Poad, Maureen Twiddy, Michelle Collinson, Victoria Hiley, Kathryn Gordon, Mark Johnson, Sunjay Jain, Aidan P Noon, Rohit Chahal, Matt Simms, Mohantha Dooldeniya, Phillip Koenig, Louise Goodwin, Julia M Brown, James W F Catto
INTRODUCTION: High-risk non-muscle invasive bladder cancer (HRNMIBC) is a heterogeneous disease that can be difficult to predict. While around 25% of cancers progress to invasion and metastases, the remaining majority of tumours remain within the bladder. It is uncertain whether patients with HRNMIBC are better treated with intravesical maintenance BCG (mBCG) immunotherapy or primary radical cystectomy (RC). A definitive randomised controlled trial (RCT) is needed to compare these two different treatments but may be difficult to recruit to and has not been attempted to date...
August 11, 2017: BMJ Open
https://www.readbyqxmd.com/read/28801407/the-boundaries-of-mild-chronic-obstructive-pulmonary-disease-copd-design-of-the-searching-clinical-copd-onset-soon-study
#18
Gonzalo Labarca, Andrea Bustamante, Gonzalo Valdivia, Rodrigo Díaz, Álvaro Huete, Paul Mac Nab, Laura Mendoza, Jaime Leppe, Carmen Lisboa, Fernando Saldías, Orlando Díaz
INTRODUCTION: Clinical onset of chronic obstructive pulmonary disease (COPD) is the point at which the disease is first identifiable by physicians. It is a poorly defined stage which seems to include both mild spirometric and non-spirometric disease, and could be described as early grade COPD, for practical purposes. While dyspnoea; chronic bronchitis and CT imaging evidence of emphysema and airway disease may be present very early, the lone significance of dyspnoea, the most relevant symptom in COPD in identifying these individuals, has been scarcely assessed...
August 11, 2017: BMJ Open
https://www.readbyqxmd.com/read/28801077/myocardial-amyloid-quantification-with-look-locker-magnetic-resonance-sequence-in-cardiac-amyloidosis-diagnostic-accuracy-in-clinical-practice-and-histological-validation
#19
Eduardo Pozo, Jose M Castellano, Anubhav Kanwar, Rajiv Deochand, Mireia Castillo-Martin, Pablo Pazos-López, Carlos González-Lengua, Keren Osman, Matthew Cham, Carlos Cordon-Cardo, Jagat Narula, Valentin Fuster, Javier Sanz
BACKGROUND: Cardiac magnetic resonance (CMR) has demonstrated its utility in the non-invasive diagnosis of cardiac amyloidosis (CA). Our aim was to evaluate the ability of standard Look-Locker sequences to quantify amyloid deposition in CA. METHODS AND RESULTS: Consecutive patients referred for CMR for possible CA were retrospectively evaluated. Positive cardiac biopsy and/or typical pattern of late gadolinium enhancement were required for the diagnosis of CA. Post-contrast T1 values were obtained from Look-Locker sequences, and correlated with markers of severity of disease and major events...
August 8, 2017: Journal of Cardiac Failure
https://www.readbyqxmd.com/read/28801026/quadruple-immunotherapy-of-bacillus-calmette-gu%C3%A3-rin-interferon-interleukin-2-and-granulocyte-macrophage-colony-stimulating-factor-as-salvage-therapy-for-non-muscle-invasive-bladder-cancer
#20
Ryan L Steinberg, Kenneth G Nepple, Kyla N Velaer, Lewis J Thomas, Michael A O'Donnell
BACKGROUND: Bacillus Calmette-Guérin (BCG) is the most effective initial intravesical therapy for high-grade non-muscle invasive bladder cancer, but many patients still fail. Combination intravesical BCG and interferon (IFN) will salvage some patients but results remain suboptimal. OBJECTIVE: We hypothesized that further immunostimulation with intravesical interleukin-2 and subcutaneous granulocyte-macrophage colony-stimulating factor may improve response to intravesical BCG and IFN in patient with prior BCG failure(s)...
August 8, 2017: Urologic Oncology
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