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Muscle Disease

Navaneetha Santhanam, Lee Kumanchik, Xiufang Guo, Frank Sommerhage, Yunqing Cai, Max Jackson, Candace Martin, George Saad, Christopher W McAleer, Ying Wang, Andrea Lavado, Christopher J Long, James J Hickman
There are currently no functional neuromuscular junction (hNMJ) systems composed of human cells that could be used for drug evaluations or toxicity testing in vitro. These systems are needed to evaluate NMJs for diseases such as amyotrophic lateral sclerosis, spinal muscular atrophy or other neurodegenerative diseases or injury states. There are certainly no model systems, animal or human, that allows for isolated treatment of motoneurons or muscle capable of generating dose response curves to evaluate pharmacological activity of these highly specialized functional units...
February 27, 2018: Biomaterials
Sana Ali Bautista, Edward J Wladis, Robert L Schultze
PURPOSE: Muller's muscle-conjunctival resection (MMCR) is a highly effective technique to correct upper eyelid ptosis. However, several authorities have raised concerns about the possibility of postoperative ocular surface dryness. This study was performed to assess the impact of MMCR on clinically meaningful parameters of the ocular surface. METHODS: In adult patients, tear break-up time, lipid layer thickness, and osmolarity were measured via direct observation, interferometry, and impedance measurements before surgery and 3 months after surgery in patients who underwent MMCR...
March 14, 2018: Ophthalmic Plastic and Reconstructive Surgery
Maria Siemionow, Joanna Cwykiel, Ahlke Heydemann, Jesus Garcia, Enza Marchese, Krzysztof Siemionow, Erzsebet Szilagyi
Duchenne Muscular Dystrophy (DMD) is a progressive and lethal disease caused by mutations of the dystrophin gene. Currently no cure exists. Stem cell therapies targeting DMD are challenged by limited engraftment and rejection despite the use of immunosuppression. There is an urgent need to introduce new stem cell-based therapies that exhibit low allogenic profiles and improved cell engraftment. In this proof-of-concept study, we develop and test a new human stem cell-based approach to increase engraftment, limit rejection, and restore dystrophin expression in the mdx/scid mouse model of DMD...
March 15, 2018: Stem Cell Reviews
Rishibha Sachdev, Karin Kappes-Horn, Lydia Paulsen, Yvonne Duernberger, Catharina Pleschka, Philip Denner, Bishwajit Kundu, Jens Reimann, Ina Vorberg
Sporadic inclusion body myositis (sIBM) is the most prevalent acquired muscle disorder in the elderly with no defined etiology or effective therapy. Endoplasmic reticulum stress and deposition of myostatin, a secreted negative regulator of muscle growth, have been implicated in disease pathology. The myostatin signaling pathway has emerged as a major target for symptomatic treatment of muscle atrophy. Here, we systematically analyzed the maturation and secretion of myostatin precursor MstnPP and its metabolites in a human muscle cell line...
March 15, 2018: Molecular Neurobiology
Kondababu Kurakula, Duco S Koenis, Mark A Herzik, Yanyun Liu, John W Craft, Pieter B van Loenen, Mariska Vos, M Khang Tran, Henri H Versteeg, Marie-José T H Goumans, Wolfram Ruf, Carlie J M de Vries, Mehmet Şen
Tissue Factor is a cell-surface glycoprotein expressed in various cells of the vasculature and is the principal regulator of the blood coagulation cascade and hemostasis. Notably, aberrant expression of Tissue Factor is associated with cardiovascular pathologies such as atherosclerosis and thrombosis. Here, we sought to identify factors that regulate Tissue Factor gene expression and activity. Tissue Factor gene expression is regulated by various transcription factors, including activating protein-1 and nuclear factor-kappa B...
March 15, 2018: Haematologica
D K Boruah, A Prakash, B B Gogoi, R R Yadav, D D Dhingani, B Sarma
BACKGROUND AND PURPOSE: Hirayama disease is a benign focal amyotrophy of the distal upper limbs involving C7, C8, and T1 segmental myotomes with sparing of the brachioradialis and proximal muscles of the upper limb innervated by C5-6 myotomes. The objective of the present study was to study the utility of MR imaging in young patients presenting with weakness and wasting of the distal upper extremity and to evaluate the importance of the laminodural space during flexion cervical MR imaging...
March 15, 2018: AJNR. American Journal of Neuroradiology
Yunlong Zou, Zhiyuan Li, Yunjing Zou, Haiyang Hao, Ning Li, Qiuyan Li
The regulatory function of Fbxo40 has been well characterized in mice. As a key component of the SCF-E3 ubiquitin ligase complex, Fbxo40 induces IRS1 ubiquitination, thus inactivating the IGF1/Akt pathway. The expression of Fbxo40 is restricted to muscle, and mice with an Fbxo40 null mutation exhibit muscle hypertrophy. However, the function of FBXO40 has not been elucidated in pigs, and it is not known whether FBXO40 mutations affect their health. We therefore generated FBXO40 knockout pigs using somatic cell nuclear transfer (SCNT) technology...
March 12, 2018: Biochemical and Biophysical Research Communications
Tereza Kmentova, Anna Valerianova, Lucie Kovarova, Jana Lachmanova, Zuzana Hladinova, Jan Malik
BACKGROUND: Creation of vascular access leads to considerable local haemodynamic changes with decreased hand perfusion. Distal limb tissues then represent a model of hand ischaemia effect on muscles. The aim of our study was to investigate how the presence of vascular access influences the hand muscle strength in end-stage renal disease patients. METHODS: We included 52 chronically haemodialysed patients with upper limb access without clinical signs of hand ischaemia...
March 1, 2018: Journal of Vascular Access
Kexin Wang, Yang Xu, Qiong Sun, Jiangang Long, Jiankang Liu, Jian Ding
The multipurpose organelle mitochondria play an essential role(s) in controlling cardiac muscle contraction. Mitochondria, not only function as the powerhouses and the energy source of myocytes, but also modulate intracellular Ca2+ homeostasis, the production of intermediary metabolites/reactive oxygen species (ROS), and other cellular processes. Those molecular events can substantially influence myocardial contraction. Mitochondrial dysfunction is usually associated with cardiac remodeling, and is the causal factor of heart contraction defects in many cases...
March 16, 2018: Free Radical Research
Elizabeth Carey
No abstract text is available yet for this article.
March 15, 2018: Liver Transplantation
Karlien Mul, Chad Heatwole, Katy Eichinger, Nuran Dilek, William B Martens, Baziel G M van Engelen, Rabi Tawil, Jeffrey M Statland
INTRODUCTION: Electrical impedance myography (EIM) is a non-invasive technique for measuring muscle composition and a potential physiological biomarker for facioscapulohumeral muscular dystrophy (FSHD). METHODS: Thirty-two genetically confirmed and clinically affected FSHD participants underwent EIM in 7 muscles bilaterally. Correlations between EIM and baseline clinical measures were used to select EIM parameters of interest in FSHD, and EIM and clinical measures were followed for 1 year...
March 15, 2018: Muscle & Nerve
Orhan Beger, Uğur Dinç, Burhan Beger, Deniz Uzmansel, Zeliha Kurtoğlu
PURPOSE: The main objective of this study is to showcase the growth dynamics of levator scapulae, rhomboid major, and rhomboid minor algebraically, to help plan surgeries on newborns and young infants. METHODS: Twenty-five formalin-fixed fetuses (11 male-14 female) with a mean gestational age of 21.80 ± 2.61 (range 18-27) weeks present in the inventory of Mersin University Faculty of Medicine Anatomy Department were dissected. Surface area of levator scapulae, rhomboid major, and rhomboid minor was calculated using digital image analysis software; width and length parameters were measured using digital calipers...
March 15, 2018: Surgical and Radiologic Anatomy: SRA
Carmelo Messina, Gabriele Maffi, Jacopo Antonino Vitale, Fabio Massimo Ulivieri, Giuseppe Guglielmi, Luca Maria Sconfienza
Osteoporosis and sarcopenia represent two major health problems with an increasing prevalence in the elderly population. The correlation between these diseases has been widely reported, leading to the development of the term "osteosarcopenia" to diagnose those patients suffering from both diseases. Several imaging methods for the diagnosis and management of osteoporosis exist, with dual-energy X-ray absorptiometry (DXA) being the most commonly used for measuring bone mineral density (BMD). Imaging technique other than DXA is represented by conventional radiography, computed tomography (CT) and ultrasound (US)...
February 2018: Quantitative Imaging in Medicine and Surgery
Sara Guerri, Daniele Mercatelli, Maria Pilar Aparisi Gómez, Alessandro Napoli, Giuseppe Battista, Giuseppe Guglielmi, Alberto Bazzocchi
Bone and muscle are two deeply interconnected organs and a strong relationship between them exists in their development and maintenance. The peak of both bone and muscle mass is achieved in early adulthood, followed by a progressive decline after the age of 40. The increase in life expectancy in developed countries resulted in an increase of degenerative diseases affecting the musculoskeletal system. Osteoporosis and sarcopenia represent a major cause of morbidity and mortality in the elderly population and are associated with a significant increase in healthcare costs...
February 2018: Quantitative Imaging in Medicine and Surgery
Jayendra M Bhalodiya, Arnab Palit, Manoj K Tiwari, Sanjay K Prasad, Sunil K Bhudia, Theodoros N Arvanitis, Mark A Williams
Cardiovascular disease diagnosis and prognosis can be improved by measuring patient-specific in-vivo local myocardial strain using Magnetic Resonance Imaging. Local myocardial strain can be determined by tracking the movement of sample muscles points during cardiac cycle using cardiac motion estimation model. The tracking accuracy of the benchmark Free Form Deformation (FFD) model is greatly affected due to its dependency on tunable parameters and regularisation function. Therefore, Hierarchical Template Matching (HTM) model, which is independent of tunable parameters, regularisation function, and image-specific features, is proposed in this article...
March 14, 2018: Scientific Reports
Didem Onk, Fatih Özçelik, Oruç Alper Onk, Murat Günay, Tülin Akarsu Ayazoğlu, Ethem Ünver
BACKGROUND The present study sought to assess the renal and liver protective effect of N-acetylcysteine through NH3 and urea metabolism in patients with chronic obstructive pulmonary disease who were scheduled for coronary artery bypass grafting surgery. MATERIAL AND METHODS Patients with chronic obstructive pulmonary disease (COPD) who were scheduled for coronary artery bypass grafting were divided into 2 groups so as to receive (Group 1, n=35) or not receive (Group 2, n=35) 900 mg/day of n-acetylcysteine for 7 days before the operation starting from their admission to the service by a pulmonologist with the purpose of treating COPD until the day of surgery...
March 15, 2018: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
Jean K Mah, Jia Feng, Marni B Jacobs, Tina Duong, Kate Carroll, Katy de Valle, Cara L Carty, Lauren P Morgenroth, Michela Guglieri, Monique M Ryan, Paula R Clemens, Mathula Thangarajh, Richard Webster, Edward Smith, Anne M Connolly, Craig M McDonald, Peter Karachunski, Mar Tulinius, Amy Harper, Avital Cnaan, Yi-Wen Chen
OBJECTIVES: To investigate motor function associations with age, sex, and D4Z4 repeats among participants with early-onset facioscapulohumeral muscular dystrophy (FSHD) type 1 as defined by weakness onset before 10 years of age. METHODS: We collected standardized motor assessments, including manual muscle testing (MMT), quantitative muscle testing, functional motor evaluations, and clinical severity scores (CSSs), at 12 Cooperative International Neuromuscular Research Group centers...
March 14, 2018: Neurology
Adam J Chicco, Catherine H Le, Erich Gnaiger, Hans C Dreyer, Jonathan B Muyskens, Angelo D'Alessandro, Travis Nemkov, Austin D Hocker, Jessica E Prenni, Lisa M Wolfe, Nathan M Sindt, Andrew T Lovering, Andrew W Subudhi, Robert C Roach
Metabolic responses to hypoxia play important roles in cell survival strategies and disease pathogenesis in humans.  However, the homeostatic adjustments that balance changes in energy supply and demand to maintain organismal function under chronic low oxygen conditions remain incompletely understood, making it difficult to distinguish adaptive from maladaptive responses in hypoxia-related pathologies.  We integrated metabolomic and proteomic profiling with mitochondrial respirometry and blood gas analyses to comprehensively define the physiological responses of skeletal muscle energy metabolism to 16 days of high-altitude hypoxia (5260 m) in healthy volunteers from the AltitudeOmics project...
March 14, 2018: Journal of Biological Chemistry
Thenappan Thenappan, Mark L Ormiston, John J Ryan, Stephen L Archer
Pulmonary hypertension is defined as a resting mean pulmonary artery pressure of 25 mm Hg or above. This review deals with pulmonary arterial hypertension (PAH), a type of pulmonary hypertension that primarily affects the pulmonary vasculature. In PAH, the pulmonary vasculature is dynamically obstructed by vasoconstriction, structurally obstructed by adverse vascular remodeling, and pathologically non-compliant as a result of vascular fibrosis and stiffening. Many cell types are abnormal in PAH, including vascular cells (endothelial cells, smooth muscle cells, and fibroblasts) and inflammatory cells...
March 14, 2018: BMJ: British Medical Journal
Ramin Sami, Raheleh Sadegh, Neda Esmailzadehha, Sanaz Mortazian, Masoomeh Nazem, Mohammadali Zohal
Malnutrition is one of the most important factors that lead to lower quality of life in patients suffering from chronic obstructive pulmonary disease (COPD). There are several methods for assessing malnutrition including anthropometric indexes. The aim of this study was to determine the association of anthropometric indexes with disease severity in male patients with COPD in Qazvin, Iran. This cross-sectional study was conducted on 72 male patients with COPD in Qazvin, Iran, from May to December 2014. Spirometry was performed for all participants...
March 1, 2018: American Journal of Men's Health
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