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https://www.readbyqxmd.com/read/28343168/potential-skin-involvement-in-als-revisiting-charcot-s-observation-a-review-of-skin-abnormalities-in-als
#1
Bastien Paré, François Gros-Louis
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting motor neurons of the brain and spinal cord, leading to progressive paralysis and death. Interestingly, many skin changes have been reported in ALS patients, but never as yet fully explained. These observations could be due to the common embryonic origin of the skin and neural tissue known as the ectodermal germ layer. Following the first observation in ALS patients' skin by Dr Charcot in the 19th century, in the absence of bedsores unlike other bedridden patients, other morphological and molecular changes have been observed...
March 25, 2017: Reviews in the Neurosciences
https://www.readbyqxmd.com/read/28342750/mutation-of-the-caspase-3-cleavage-site-in-the-astroglial-glutamate-transporter-eaat2-delays-disease-progression-and-extends-lifespan-in-the-sod1-g93a-mouse-model-of-als
#2
Lauren Taylor Rosenblum, Shashirekha Shamamandri-Markandaiah, Biswarup Ghosh, Emily Foran, Angelo C Lepore, Piera Pasinelli, Davide Trotti
Downregulation in the astroglial glutamate transporter EAAT2 in amyotrophic lateral sclerosis (ALS) patients and mutant SOD1 mouse models of ALS is believed to contribute to the death of motor neurons by excitotoxicity. We previously reported that caspase-3 cleaves EAAT2 at a unique cleavage consensus site located in its c-terminus domain, a proteolytic cleavage that also occurs in vivo in the mutant SOD1 mouse model of ALS and leads to accumulation of a sumoylated EAAT2 C-Terminus fragment (CTE-SUMO1) beginning around onset of disease...
March 22, 2017: Experimental Neurology
https://www.readbyqxmd.com/read/28340431/utility-of-repetitive-nerve-stimulation-test-for-als-diagnosis
#3
Yuki Hatanaka, Mana Higashihara, Takashi Chiba, Yosuke Miyaji, Yasuomi Kawamura, Masahiro Sonoo
OBJECTIVE: Decremental responses in the repetitive nerve stimulation (RNS) test in amyotrophic lateral sclerosis (ALS) patients have been reported, although their possible diagnostic role has received little investigation. We investigated their diagnostic role in differentiation between ALS and cervical spondylotic amyotrophy (CSA), an important ALS mimic especially in Japan. METHODS: Patients were prospectively enrolled and the diagnosis was confirmed by follow-up...
March 6, 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28338889/measuring-occupational-exposure-to-extremely-low-frequency-electric-fields-at-220-kv-substations
#4
Herkko Pirkkalainen, Timo Heiskanen, Juhani Tonteri, Jarmo Elovaara, Penttilä Mika, Leena Korpinen
Earlier studies conducted at 400 and 110 kV substations in Finland have shown that the occupational exposure to electric fields can exceed the action levels (ALs) set by Directive 2013/35/EU. This is a case study investigating the level of occupational exposure experienced by workers at 220 kV substations in order to determine if the actions levels are being exceeded. The measurements were conducted at two old 220 kV substations in Finland. The higher AL of 20 kV m-1 was exceeded at both substations.
March 3, 2017: Radiation Protection Dosimetry
https://www.readbyqxmd.com/read/28337659/a20-in-multiple-sclerosis-and-parkinson-s-disease-clue-to-a-common-dysregulation-of-anti-inflammatory-pathways
#5
Simona Perga, Serena Martire, Francesca Montarolo, Nicole D Navone, Andrea Calvo, Giuseppe Fuda, Alberto Marchet, Daniela Leotta, Adriano Chiò, Antonio Bertolotto
Chronic inflammation significantly contributes to the pathogenesis of several neurodegenerative disorders. In physiological conditions, a chronic inflammatory state is prevented through the termination of the acute inflammatory response once the triggering insult is eliminated. Several mechanisms regulate the resolution of inflammation. Among these, a potent inhibitor of the pro-inflammatory NF-kB signaling known as A20 has emerged as a key player. Recent studies have shown reduced blood levels of A20 in the patients of diverse chronic inflammatory diseases...
March 23, 2017: Neurotoxicity Research
https://www.readbyqxmd.com/read/28337565/blood-trace-metals-in-a-sporadic-amyotrophic-lateral-sclerosis-geographical-cluster
#6
Stefano De Benedetti, Giorgio Lucchini, Cristian Del Bò, Valeria Deon, Alessandro Marocchi, Silvana Penco, Christian Lunetta, Elisabetta Gianazza, Francesco Bonomi, Stefania Iametti
Amyotrophic lateral sclerosis (ALS) is a fatal disorder with unknown etiology, in which genetic and environmental factors interplay to determine the onset and the course of the disease. Exposure to toxic metals has been proposed to be involved in the etiology of the disease either through a direct damage or by promoting oxidative stress. In this study we evaluated the concentration of a panel of metals in serum and whole blood of a small group of sporadic patients, all living in a defined geographical area, for which acid mine drainage has been reported...
March 23, 2017: Biometals: An International Journal on the Role of Metal Ions in Biology, Biochemistry, and Medicine
https://www.readbyqxmd.com/read/28337412/differential-involvement-of-corticospinal-tract-cst-fibers-in-umn-predominant-als-patients-with-or-without-cst-hyperintensity-a-diffusion-tensor-tractography-study
#7
Venkateswaran Rajagopalan, Erik P Pioro
Diagnosis of amyotrophic lateral sclerosis (ALS) depends on clinical evidence of combined upper motor neuron (UMN) and lower motor neuron (LMN) degeneration, although ALS patients can present with features predominantly of one or the other. Some UMN-predominant patients show hyperintense signal along the intracranial corticospinal tract (CST) on T2- and proton density (PD)-weighted images (ALS-CST +), and appear to have faster disease progression when compared to those without CST hyperintensity (ALS-CST -)...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28337334/correction-to-chiral-cyclohexane-1-3-diones-as-inhibitors-of-mutant-sod1-dependent-protein-aggregation-for-the-treatment-of-als
#8
Yinan Zhang, Radhia Benmohamed, Wei Zhang, Jinho Kim, Christina K Edgerly, Yaoqiu Zhu, Richard I Morimoto, Robert J Ferrante, Donald R Kirsch, Richard B Silverman
[This corrects the article DOI: 10.1021/ml3000963.].
March 9, 2017: ACS Medicinal Chemistry Letters
https://www.readbyqxmd.com/read/28337172/bone-marrow-derived-cell-accumulation-in-the-spinal-cord-is-independent-of-peripheral-mobilization-in-a-mouse-model-of-amyotrophic-lateral-sclerosis
#9
Kyle Peake, John Manning, Coral-Ann Lewis, Kevin Tran, Fabio Rossi, Charles Krieger
Bone marrow-derived cells (BMDCs) are capable of migrating across the blood-brain barrier (BBB) and accumulating in the central nervous system (CNS) when transplanted into recipients conditioned with whole-body irradiation or chemotherapy. We used the chemotherapeutic agents busulfan and treosulfan to condition recipient mice for transplantation with bone marrow (BM) cells isolated from donor mice ubiquitously expressing green fluorescent protein. We attempted to increase the accumulation of BMDCs in the CNS by mobilization of BMDCs using either, or both, granulocyte colony-stimulating factor (GCSF) or plerixafor (AMD3100)...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28337023/reconstruction-of-large-scale-defects-with-a-novel-hybrid-scaffold-made-from-poly-l-lactic-acid-nanohydroxyapatite-alendronate-loaded-chitosan-microsphere-in-vitro-and-in-vivo-studies
#10
Hongwei Wu, Pengfei Lei, Gengyan Liu, Yu Shrike Zhang, Jingzhou Yang, Longbo Zhang, Jie Xie, Wanting Niu, Hua Liu, Jianming Ruan, Yihe Hu, Chaoyue Zhang
A chitosan-based microsphere delivery system has been fabricated for controlled release of alendronate (AL). The present study aimed to incorporate the chitosan/hydroxyapatite microspheres-loaded with AL (CH/nHA-AL) into poly(L-lactic acid)/nanohydroxyapatite (PLLA/nHA) matrix to prepare a novel microspheres-scaffold hybrid system (CM-ALs) for drug delivery and bone tissue engineering application. The characteristics of CM-ALs scaffolds containing 10% and 20% CH/nHA-AL were evaluated in vitro, including surface morphology and porosity, mechanical properties, drug release, degradation, and osteogenic differentiation...
March 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28336525/extracellular-tdp-43-aggregates-target-mapk-mak-mrk-overlapping-kinase-mok-and-trigger-caspase-3-il-18-signaling-in-microglia
#11
María M Leal-Lasarte, Jaime M Franco, Adahir Labrador-Garrido, David Pozo, Cintia Roodveldt
Dysregulated microglial responses are central in neurodegenerative proteinopathies, including amyotrophic lateral sclerosis (ALS) and frontotemporal lobar disease (FTLD). Pathologic TDP-43, which is typically found in intracellular inclusions, is a misfolding protein with emerging roles in ALS and FTLD. Recently, TDP-43 species have been found in extracellular fluids of patients; however, the overall implications of TDP-43-mediated signaling linked to neuroinflammation are poorly understood. Our work-the first, to our knowledge, to focus on innate immunity responses to TDP-43 aggregates-shows that such species are internalized by microglia and cause abnormal mobilization of endogenous TDP-43...
March 23, 2017: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/28335486/implicit-regularization-for-reconstructing-3d-building-rooftop-models-using-airborne-lidar-data
#12
Jaewook Jung, Yoonseok Jwa, Gunho Sohn
With rapid urbanization, highly accurate and semantically rich virtualization of building assets in 3D become more critical for supporting various applications, including urban planning, emergency response and location-based services. Many research efforts have been conducted to automatically reconstruct building models at city-scale from remotely sensed data. However, developing a fully-automated photogrammetric computer vision system enabling the massive generation of highly accurate building models still remains a challenging task...
March 19, 2017: Sensors
https://www.readbyqxmd.com/read/28335033/lingual-alveolar-contact-pressure-during-speech-in-amyotrophic-lateral-sclerosis-preliminary-findings
#13
Jeff Searl, Stephanie Knollhoff, Richard J Barohn
Purpose: This preliminary study on lingual-alveolar contact pressures (LACP) in people with amyotrophic lateral sclerosis (ALS) had several aims: (a) to evaluate whether the protocol induced fatigue, (b) to compare LACP during speech (LACP-Sp) and during maximum isometric pressing (LACP-Max) in people with ALS (PALS) versus healthy controls, (c) to compare the percentage of LACP-Max utilized during speech (%Max) for PALS versus controls, and (d) to evaluate relationships between LACP-Sp and LACP-Max with word intelligibility...
March 23, 2017: Journal of Speech, Language, and Hearing Research: JSLHR
https://www.readbyqxmd.com/read/28334913/tdp-43-mutations-causing-amyotrophic-lateral-sclerosis-are-associated-with-altered-expression-of-rna-binding-protein-hnrnp-k-and-affect-the-nrf2-antioxidant-pathway
#14
Diane Moujalled, Alexandra Grubman, Karla Acevedo, Shu Yang, Yazi D Ke, Donia M Moujalled, Clare Duncan, Aphrodite Caragounis, Nirma D Perera, Bradley J Turner, Mercedes Prudencio, Leonard Petrucelli, Ian Blair, Lars M Ittner, Peter J Crouch, Jeffrey R Liddell, Anthony R White
TAR DNA binding protein 43 (TDP-43) is a major disease-associated protein involved in the pathogenesis of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U). Our previous studies found a direct association between TDP-43 and heterogeneous nuclear ribonucleoprotein K (hnRNP K). In this study, utilizing ALS patient fibroblasts harboring a TDP-43M337V mutation and NSC-34 motor neuronal cell line expressing TDP-43Q331K mutation, we show that hnRNP K expression is impaired in urea soluble extracts from mutant TDP-43 cell models...
March 9, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28334804/a-critical-role-of-hrd1-in-the-regulation-of-optineurin-degradation-and-aggresome-formation
#15
Jiahui Mao, Qin Xia, Chunfeng Liu, Zheng Ying, Hongfeng Wang, Guanghui Wang
Mutations in optineurin (OPTN) are associated with several human disorders including amyotrophic lateral sclerosis (ALS) and primary open-angle glaucoma (POAG). OPTN is known to be a multifunctional autophagy receptor that plays important roles in NF-κB signaling, vesicle trafficking, maintenance of the Golgi apparatus and autophagy. Given that a loss of neurons and an abnormal aggregation of disease proteins are two key features of neurodegenerative diseases, protein quality control systems are considered to be tightly associated with neurodegeneration...
March 11, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28334553/a-multi-matrix-hilic-ms-ms-method-for-the-quantitation-of-endogenous-small-molecule-neurological-biomarker-n-acetyl-aspartic-acid-naa
#16
Dewakar Sangaraju, Sheerin K Shahidi-Latham, Braydon L Burgess, Brian Dean, Xiao Ding
A multi-matrix hydrophilic interaction liquid chromatography tandem mass spectrometric method (HILIC-MS/MS) was developed for the quantitation of N-Acetyl Aspartic acid (NAA) using stable isotope labeled internal standard, D3-NAA in various biological matrices such as human plasma, human CSF, mouse plasma, brain and spinal cord. A high throughput 96-well plate format supported liquid extraction (SLE) procedure was developed and used for sample preparation. Mass spectrometric analysis of NAA was performed using selected reaction monitoring transitions in positive electrospray ionization mode...
March 14, 2017: Journal of Pharmaceutical and Biomedical Analysis
https://www.readbyqxmd.com/read/28332094/cryptic-exon-incorporation-occurs-in-alzheimer-s-brain-lacking-tdp-43-inclusion-but-exhibiting-nuclear-clearance-of-tdp-43
#17
Mingkuan Sun, William Bell, Katherine D LaClair, Jonathan P Ling, Heather Han, Yusuke Kageyama, Olga Pletnikova, Juan C Troncoso, Philip C Wong, Liam L Chen
Abnormal accumulation of TDP-43 into cytoplasmic or nuclear inclusions with accompanying nuclear clearance, a common pathology initially identified in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), has also been found in Alzheimer' disease (AD). TDP-43 serves as a splicing repressor of nonconserved cryptic exons and that such function is compromised in brains of ALS and FTD patients, suggesting that nuclear clearance of TDP-43 underlies its inability to repress cryptic exons. However, whether TDP-43 cytoplasmic aggregates are a prerequisite for the incorporation of cryptic exons is not known...
March 22, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28332040/edinburgh-cognitive-and-behavioural-als-screen-ecas-italian-version-regression-based-norms-and-equivalent-scores
#18
Mattia Siciliano, Luigi Trojano, Francesca Trojsi, Roberta Greco, Manuela Santoro, Giuseppe Basile, Fausta Piscopo, Alfonsina D'Iorio, Manila Patrone, Cinzia Femiano, Mariarosaria Monsurrò, Gioacchino Tedeschi, Gabriella Santangelo
Cognitive assessment for individuals with Amyotrophic Lateral Sclerosis (ALS) can be difficult because of frequent occurrence of difficulties with speech, writing, and drawing. The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) is a recent multi-domain neuropsychological screening tool specifically devised for this purpose, and it assesses the following domains: executive functions, social cognition, verbal fluency and language (ALS-specific), but also memory and visuospatial abilities (Non-ALS specific)...
March 22, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/28331770/traditional-food-items-in-ogimi-okinawa-l-serine-content-and-the-potential-for-neuroprotection
#19
REVIEW
Paul Alan Cox, James S Metcalf
PURPOSE OF REVIEW: Ogimi village is renowned for its aging population. We sought to determine if the l-serine content of their diet could account for their neurological health. RECENT FINDINGS: The most frequently consumed food items, including tofu and seaweeds, are rich in the dietary amino acid l-serine. l-serine content of the Ogimi diet >8 grams/day for Ogimi women significantly exceeds the average American dietary intake of 2.5 grams/day for women >70 years old...
2017: Current Nutrition Reports
https://www.readbyqxmd.com/read/28331009/the-modulation-of-two-motor-behaviours-by-persistent-sodium-currents-in-xenopus-laevis-tadpoles
#20
Erik Svensson, Hugo Jeffreys, Wen-Chang Li
Persistent sodium currents (INaP) are common in neuronal circuitries and they have been implicated in several diseases like amyotrophic lateral sclerosis (ALS) and epilepsy. However, the role of INaP in the regulation of specific behaviours is still poorly understood. Here we have characterized INaP and investigated its role in the swimming and struggling behaviour of Xenopus tadpoles. INaP was identified in three groups of neurons, namely sensory Rohon-Beard neurons (RB neurons), descending interneurons (dINs), and non-dINs (neurons rhythmically active in swimming)...
March 22, 2017: Journal of Neurophysiology
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