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Tanmay Mahapatra, Kaushik Chakraborty, Sanchita Mahapatra, Umakanta Mahapatra, Naren Pandey, Peter L Thomson, Arthur W Musk, Ramendra N Mitra
BACKGROUND: Progressive burden of diabetes mellitus is a major concern in India. Data on the predictors of poor glycemic control among diabetics are scanty. A population-based cross-sectional study nested in an urban cohort was thus conducted in West Bengal, India to determine the burden and correlates of total and uncontrolled abnormalities in glucose metabolism (AGM) in a representative population. METHODS: From 9046 adult cohort-members, 269 randomly selected consenting subjects (non-response = 7...
2016: PloS One
Mouna Necibi, Laurent Lanceleur, Nadia Mzoughi, Mathilde Monperrus
A new analytical method for the simultaneous determination of eight synthetic musks compounds (SMs) including five polycyclic musks (PCMs) and three nitro musks (NMs) was validated for sediment samples based on a simple QuEChERS (Quick, Easy, Cheap, Effective, Rugged and Safe) sample preparation procedure followed by gas chromatography-mass spectrometry (GC-MS). Good analytical performances were obtained for all the target compounds. For the validation of the method, internal calibration (IC) and internal calibration with QuEChERS (ICQ) were compared...
October 14, 2016: Bulletin of Environmental Contamination and Toxicology
Daniel B Drachman
The basic abnormality in myasthenia gravis (MG) is a reduction in acetylcholine receptors (AChRs) at neuromuscular junctions due to the effects of autoantibodies that are directed against the AChRs in most patients, or against neighboring proteins involved in the clustering of AChRs (MuSK, LRP-4, or agrin). Clinically, MG is characterized by muscle weakness and fatigue, often in typical patterns. The diagnosis may be missed early, and depends on the recognition of clinical manifestations, the measurement of autoantibodies, and/or electrophysiological features...
October 2016: Seminars in Neurology
Lukas Kirzinger, Andrei Khomenko, Wilhelm Schulte-Mattler, Roland Backhaus, Sabine Platen, Berthold Schalke
BACKGROUND: Adult and pediatric patients suffering from MuSK (muscle-specific kinase) -antibody positive myasthenia gravis exhibit similar features to individuals with acetylcholine receptor (AChR) antibodies, but they differ in several characteristics such as a predominant bulbar, respiratory and neck weakness, a generally worse disease severity and a tendency to develop muscle atrophy. Muscle atrophy is a rare phenomenon that is usually restricted to the facial muscles. RESULTS: We describe a girl with MuSK-antibody positive myasthenia gravis who developed a myopathy with severe generalized muscular weakness, muscle atrophy, and myopathic changes on electromyography...
August 20, 2016: Pediatric Neurology
Carrie A McDonough, Paul A Helm, Derek C G Muir, Gavino Puggioni, Rainer Lohmann
Polycyclic musks (PCMs) are synthetic fragrance compounds used in personal care products and household cleaners. Previous studies have indicated that PCMs are introduced to aquatic environments via wastewater and river discharge. Polyethylene passive samplers (PEs) were deployed in air and water during winter 2011 and summer 2012 to investigate the role of population centers as sources of these contaminants to the Great Lakes and determine whether the lakes were acting as sources of PCMs via volatilization...
September 28, 2016: Environmental Science & Technology
B Z Camurdanoglu, C Hrovat, G Dürnberger, M Madalinski, K Mechtler, R Herbst
The neuromuscular junction (NMJ) forms when a motor neuron contacts a muscle fibre. A reciprocal exchange of signals initiates a cascade of signalling events that result in pre- and postsynaptic differentiation. At the centre of these signalling events stands muscle specific kinase (MuSK). MuSK activation, kinase activity and subsequent downstream signalling are crucial for NMJ formation as well as maintenance. Therefore MuSK kinase activity is tightly regulated to ensure proper NMJ development. We have identified a novel serine phosphorylation site at position 751 in MuSK that is increasingly phosphorylated upon agrin stimulation...
September 26, 2016: Scientific Reports
Claudia Ferrario, Antonio Finizio, Sara Villa
Meltwater samples collected in early and late summer from three Alpine glaciers were analysed to determine the occurrence of POPs (Persistent Organic Pollutants: DDTs, HCHs and PCBs) and emerging contaminants (current used pesticides and polycyclic musk fragrances). For legacy POPs, we reconstructed a concentration time series using data from previous surveys in the same areas (starting from 2000). The results suggest a declining tendency of these compounds, probably related to the introduction of international regulations, which has led the strong use reduction and ban of these compounds...
September 15, 2016: Science of the Total Environment
John A Rudd, Man P Ngan, Zengbing Lu, Guy A Higgins, Claudio Giuliano, Emanuela Lovati, Claudio Pietra
BACKGROUND AND AIMS: Chemotherapy-induced acute and delayed emesis involves the activation of multiple pathways, with 5-hydroxytryptamine (5-HT; serotonin) playing a major role in the initial response. Substance P tachykinin NK1 receptor antagonists can reduce emesis induced by disparate emetic challenges and therefore have a clinical utility as broad inhibitory anti-emetic drugs. In the present studies, we investigate the broad inhibitory anti-emetic profile of a relatively new NK1 receptor antagonist, netupitant, alone or in combination with the long acting 5-HT3 receptor antagonist, palonosetron, for a potential to reduce emesis in ferrets and shrews...
2016: Frontiers in Pharmacology
Li Yao, Jian-Liang Zhao, You-Sheng Liu, Yuan-Yuan Yang, Wang-Rong Liu, Guang-Guo Ying
A sensitive and selective quick, easy, cheap, effective, rugged, and safe (QuEChERS) extraction combined with dispersive solid-phase extraction (d-SPE) cleanup method was developed to simultaneously extract a wide range of personal care products (16 biocides, 4 synthetic musks, and 4 benzotriazoles) in fish muscle and liver tissues. In order to get satisfactory recoveries, different extraction parameters were optimized, including extraction salts and d-SPE materials, extraction solvents and acetic acid contents in organic phase, and the ratios of solvent and water...
November 2016: Analytical and Bioanalytical Chemistry
Yu Hong, Hai-Feng Li, Geir Olve Skeie, Fredrik Romi, Hong-Jun Hao, Xu Zhang, Xiang Gao, Jone Furlund Owe, Nils Erik Gilhus
Myasthenia gravis (MG) is an autoimmune disorder with heterogeneity. Antibodies against acetylcholine receptor (AChR), muscle-specific kinase (MuSK), titin and ryanodine receptor (RyR) were examined in 437 adult Chinese MG patients. The AChR, MuSK, titin and RyR antibodies were found in 82.2%, 2.3%, 28.4% and 23.8% of all patients. Autoantibody profiles vary among different MG subgroups. Thymoma MG patients had high frequencies of AChR (99.2%), titin (50.8%) and RyR antibodies (46.9%). The titin and RyR antibodies also showed high frequencies in late onset patients (54...
September 15, 2016: Journal of Neuroimmunology
Atilgan Yilmaz, Chandramohan Kattamuri, Rana N Ozdeslik, Carolyn Schmiedel, Sarah Mentzer, Christoph Schorl, Elena Oancea, Thomas B Thompson, Justin R Fallon
Bone morphogenetic proteins (BMPs) function in most tissues but have cell type-specific effects. Given the relatively small number of BMP receptors, this exquisite signaling specificity requires additional molecules to regulate this pathway's output. The receptor tyrosine kinase MuSK (muscle-specific kinase) is critical for neuromuscular junction formation and maintenance. Here, we show that MuSK also promotes BMP signaling in muscle cells. MuSK bound to BMP4 and related BMPs with low nanomolar affinity in vitro and to the type I BMP receptors ALK3 and ALK6 in a ligand-independent manner both in vitro and in cultured myotubes...
2016: Science Signaling
Yanpeng Gao, Yuemeng Ji, Guiying Li, Bixian Mai, Taicheng An
Polycyclic musks (PCMs) have recently caused a worldwide environmental concern due to their bioaccumulation potential and ecotoxicological effects. Herein, the OH-initiated indirect photochemical transformation mechanism, environmental fate and ecotoxicity of PCMs (by taking tonalide as an example) were theoretically studied. Results show that tonalide can be degraded readily through OH-addition and H-abstraction pathways, with total rate constants of 6.03 × 10(9)-15.8 × 10(9) M(-1) s(-1). The OH-addition pathways were dominant at low temperature (<∼287 K), whereas H-abstraction was the dominant pathway at high temperature...
November 15, 2016: Water Research
Xinghua Luan, Wotu Tian, Li Cao
OBJECTIVES: To describe the clinical and genetic features of a Chinese congenital myasthenic syndromes (CMS) patient with two novel missense mutations in muscle specific receptor tyrosine kinase (MUSK) gene and review 15 MUSK-related CMS patients from 8 countries. METHODS: The patient was a 30-year-old man with chronic progressively proximal limb weakness for 22 years and diagnosed as muscular dystrophy before. Serum creatine kinase (CK) was normal. Repetitive nerve stimulation (RNS) test showed decrements at low rate stimulation...
November 2016: Clinical Neurology and Neurosurgery
Qian Wang, Barry C Kelly
This study involved a comprehensive thirteen month survey of synthetic musks, triclosan (TCS) and methyl triclosan (MTCS) in surface water, as well as suspended particular matter (SPM) and bottom sediments in a tropical urban catchment in Singapore. The polycyclic musk, Galaxolide (HHCB), exhibited the highest concentration among musk compounds, ranging from 5.16 to 42.9ng/L in surface water, 11.0 to 108ng/g dry wt. in sediments and 44.1 to 81.3ng/g dry wt. in SPM. Concentrations of musk ketone, the dominant nitroaromatic musk, ranged from 0...
August 29, 2016: Science of the Total Environment
William Dalkin, Thomas Taetzsch, Gregorio Valdez
The neuromuscular junction (NMJ) undergoes deleterious structural and functional changes as a result of aging, injury and disease. Thus, it is imperative to understand the cellular and molecular changes involved in maintaining and repairing NMJs. For this purpose, we have developed a method to reliably and consistently examine regenerating NMJs in mice. This nerve injury method involves crushing the common fibular nerve as it passes over the lateral head of the gastrocnemius muscle tendon near the knee. Using 70 day old female mice, we demonstrate that motor axons begin to reinnervate previous postsynaptic targets within 7 days post-crush...
2016: Journal of Visualized Experiments: JoVE
Nese Sinmaz, Tina Nguyen, Fiona Tea, Russell C Dale, Fabienne Brilot
BACKGROUND: Our knowledge of autoantibody-associated diseases of the central (CNS) and peripheral (PNS) nervous systems has expanded greatly over the recent years. A number of extracellular and intracellular autoantigens have been identified, and there is no doubt that this field will continue to expand as more autoantigens are discovered as a result of improved clinical awareness and methodological practice. In recent years, interest has shifted to uncover the target epitopes of these autoantibodies...
2016: Journal of Neuroinflammation
Jae-Yun Lee, Panos Stathopoulos, Sasha Gupta, Jason M Bannock, Richard J Barohn, Elizabeth Cotzomi, Mazen M Dimachkie, Leslie Jacobson, Casey S Lee, Henner Morbach, Luis Querol, Jing-Li Shan, Jason A Vander Heiden, Patrick Waters, Angela Vincent, Richard J Nowak, Kevin C O'Connor
OBJECTIVE: Myasthenia gravis (MG) is an autoimmune condition in which neurotransmission is impaired by binding of autoantibodies to acetylcholine receptors (AChR) or, in a minority of patients, to muscle specific kinase (MuSK). There are differences in the dominant IgG subclass, pathogenic mechanisms, and treatment responses between the two MG subtypes (AChR or MuSK). The antibodies are thought to be T-cell dependent, but the mechanisms underlying their production are not well understood...
June 2016: Annals of Clinical and Translational Neurology
P Franklin, H Alfonso, A Reid, N Olsen, K B Shilkin, F Brims, N de Klerk, A W Musk
BACKGROUND: Malignant mesothelioma (MM) has distinct histological subtypes (epithelioid, sarcomatoid and biphasic) with variable behaviour and prognoses. It is well recognised that survival time varies with the histological subtype of MM. It is not known, however, if asbestos exposure characteristics (type of asbestos, degree of exposure) are associated with different histological subtypes. AIM: To determine if the pathological MM subtype is associated with the type of asbestos or the attributes of asbestos exposure...
November 2016: Occupational and Environmental Medicine
Yadanar Moe, Tomoko Tanaka, Masahiro Morishita, Ryoko Ohata, Chihiro Nakahara, Takaharu Kawashima, Fumihiko Maekawa, Ichiro Sakata, Takafumi Sakai, Shinji Tsukahara
The medial preoptic nucleus (MPN) and the bed nucleus of the stria terminalis (BNST) of mice contain sexually dimorphic nuclei (SDNs) that are larger and have more neurons expressing calbindin D-28K (CB), a calcium-binding protein, in males than females. However, it is largely unknown whether such SDNs exist in species other than rodents. In this study, we performed an immunohistochemical study of CB in the MPN and BNST of musk shrews and Japanese quails to examine the existence of homologs of SDNs in mice...
September 19, 2016: Neuroscience Letters
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