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https://www.readbyqxmd.com/read/28742871/comprehensive-immune-profiling-reveals-substantial-immune-system-alterations-in-a-subset-of-patients-with-amyotrophic-lateral-sclerosis
#1
Michael P Gustafson, Nathan P Staff, Svetlana Bornschlegl, Greg W Butler, Mary L Maas, Mohamed Kazamel, Adeel Zubair, Dennis A Gastineau, Anthony J Windebank, Allan B Dietz
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease with a median lifespan of 2-3 years after diagnosis. There are few meaningful treatments that alter progression in this disease. Preclinical and clinical studies have demonstrated that neuroinflammation may play a key role in the progression rate of ALS. Despite this, there are no validated biomarkers of neuroinflammation for use in clinical practice or clinical trials. Biomarkers of neuroinflammation could improve patient management, provide new therapeutic targets, and possibly help stratify clinical trial selection and monitoring...
2017: PloS One
https://www.readbyqxmd.com/read/28741707/photophysical-and-photochemical-properties-of-naturally-cccurring-normelinonine-f-and-melinonine-f-alkaloids-and-structurally-related-n-2-and-or-n-9-methyl-%C3%AE-carboline-derivatives
#2
Federico A O Rasse-Suriani, Fernando S García-Einschlag, Matías Rafti, Tobías Schmidt De León, Pedro M David Gara, Rosa Erra-Balsells, Franco M Cabrerizo
In the present work, we have synthesized and fully characterized the photophysical and photochemical properties of a selected group of N-methyl-β-carboline derivatives (9-methyl-β-carbolines and iodine salts of 2-methyl- and 2,9-dimethyl-β-carbolinium) in aqueous solutions, in the pH-range 4.0 - 14.5. Moreover, despite the quite extensive studies reported in the literature regarding the overall photophysical behaviour of N-unsubstituted βCs, this work constitutes the first full and unambiguous characterization of anionic species of N-unsubstituted βCs (norharmane, harmane and harmine), present in aqueous solution under highly alkaline conditions (pH > 13...
July 25, 2017: Photochemistry and Photobiology
https://www.readbyqxmd.com/read/28741375/comparison-of-slow-and-forced-vital-capacities-on-ability-to-predict-survival-in-als
#3
Susana Pinto, Mamede de Carvalho
INTRODUCTION: Slow (SVC) and forced (FVC) vital capacities are the most used pulmonary function tests in amyotrophic lateral sclerosis (ALS). It is unknown if they equally predict survival in ALS. The aim of the present study was to compare both measures in predicting survival in this disease. METHODS: Consecutive definite/probable ALS patients (2000-2014) in whom respiratory tests were performed at baseline and four months later were included. All patients were evaluated with the revised ALS functional rating scale (ALSFRS-R), respiratory (RofALSFRS-R), bulbar (ALSFRSb), upper and lower limb subscores, SVC, FVC, maximal inspiratory (MIP) and expiratory (MEP) pressures...
July 25, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28741116/application-of-multivariate-curve-resolution-alternate-least-square-technique-on-extracting-pure-spectral-components-from-multiple-emitting-systems-a-case-study
#4
Madhumita Tarai, Ashok Kumar Mishra
Multiple emitting components in a fluorophoric system often produce complicated emission spectra. Extracting the individual spectral information from the composite spectra is important in order to comprehend the photophysical processes occurring in the multifluorophoric systems. Although the combination of Principal Component Analysis and Multivariate Curve Resolution-Alternate Least Square (PCA/MCR-ALS) technique is a well-known approach for curve deconvolution, its applicability in the spectral deconvolution of vibronically and electronically mixed up emitting systems as well as systems merged up with multiple electronic bands without a priori knowledge of the individual emitting species, is not properly studied...
July 24, 2017: Journal of Fluorescence
https://www.readbyqxmd.com/read/28738414/impact-of-amyotrophic-lateral-sclerosis-on-slow-tonic-myofiber-composition-in-human-extraocular-muscles
#5
Anton E Tjust, Adam Danielsson, Peter M Andersen, Thomas Brännström, Fatima Pedrosa Domellöf
Purpose: To analyze the proportion and cross-sectional area of myofibers containing myosin heavy chain slow-twitch (MyHCI) and myosin heavy chain slow tonic (MyHCsto) in extraocular muscles of autopsied amyotrophic lateral sclerosis (ALS) patients with either spinal or bulbar site of disease onset. Methods: Whole-muscle cross sections from the middle portion of the medial rectus were labeled with antibodies against MyHCI or MyHCsto and laminin. Myofibers labeled with the MyHC antibodies (MyHCI+sto) and the total number of myofibers were quantified in the orbital and global layer of 6 control individuals and 18 ALS patients...
July 1, 2017: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/28738334/time-course-of-radiological-imaging-and-variable-interindividual-symptoms-in-amyotrophic-lateral-sclerosis-and-frontotemporal-dementia-associated-with-p-arg487his-mutation-in-the-vcp%C3%A2-gene
#6
Makito Hirano, Yuko Yamagishi, Satoshi Yanagimoto, Kazumasa Saigoh, Yusaku Nakamura, Susumu Kusunoki
To our knowledge, this is the first study to report the time course of radiological imaging of 3 patients from 2 families with VCP-related amyotrophic lateral sclerosis (ALS) and dementia. Both families shared the same p.Arg487His mutation in the VCP gene encoding valosin-containing protein. The first patient started to have a typical form of ALS, followed by dementia 7 years later. The second patient, a brother of the first one, had frontotemporal dementia and parkinsonism. The third patient had simultaneous ALS and dementia...
July 24, 2017: European Neurology
https://www.readbyqxmd.com/read/28737870/analysis-of-acid-labile-subunit-and-its-usefulness-in-pediatrics
#7
REVIEW
Verónica E Zaidman
The acid-labile subunit (ALS) is an 85 kDa glycoprotein that belongs to the leucine-rich repeat superfamily. It mainly circulates in serum bound to a high molecular weight ternary complex. The main and most widely studied function of ALS is to prolong the half-life of the binary complex formed by insulin-like growth factors type 1 and 2 and its transport proteins 3 and 5. ALS serum levels are lower in neonates, reach a peak in late puberty, and then slowly decrease throughout adulthood. ALS deficiency has consequences on growth, hydrocarbon and bone metabolism, and, in some cases, it affects pubertal development...
August 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28737506/microglia-and-c9orf72-in-neuroinflammation-and-als-and-frontotemporal-dementia
#8
Deepti Lall, Robert H Baloh
Amyotrophic lateral sclerosis (ALS) is a degenerative disorder that is characterized by loss of motor neurons and shows clinical, pathological, and genetic overlap with frontotemporal dementia (FTD). Activated microglia are a universal feature of ALS/FTD pathology; however, their role in disease pathogenesis remains incompletely understood. The recent discovery that ORF 72 on chromosome 9 (C9orf72), the gene most commonly mutated in ALS/FTD, has an important role in myeloid cells opened the possibility that altered microglial function plays an active role in disease...
July 24, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28737194/-face-protective-patches-do-not-reduce-facial-pressure-ulcers-in-a-simulated-model-of-non-invasive-ventilation
#9
Hugo Riquelme M, David Wood V, Santiago Martínez F, Fernando Carmona M, Axel Peña V, Adriana Wegner A
Noninvasive ventilation (NIV) frequently involves the development of facial pressure ulcers (FPU). Its prevention considers the empirical use of protective patches between skin and mask, in order to reduce the pressure exerted by it. OBJECTIVES: To evaluate the effect of protective patches on the pressure exerted by the facial mask, and its impact on the programmed ventilatory parameters. METHOD: Bilevel NIV simulated model using full face mask in phantom with a physiological airway (ALS PRO +) in supine position...
June 2017: Revista Chilena de Pediatría
https://www.readbyqxmd.com/read/28735795/accuracy-of-2-endodontic-rotary-motors-with-integrated-apex-locator
#10
Alessandra Timponi Goes Cruz, Caroline Wichnieski, Everdan Carneiro, Ulisses Xavier da Silva Neto, Gianluca Gambarini, Lucila Piasecki
INTRODUCTION: The purpose of this study was to evaluate in vitro the efficacy of both the electronic apex locator (EAL) and auto apical reverse (AAR) functions of the endodontic motor MM Control (Micro-Mega, Besançon Cedex, France) compared with Root ZX II (J Morita, Tokyo, Japan). METHODS: After cervical preflaring, the actual lengths (ALs) of 36 single-rooted teeth were obtained up to the apical foramen. The EAL measurements at the marks "APEX" and "0.5" of both devices were obtained using an alginate model...
July 20, 2017: Journal of Endodontics
https://www.readbyqxmd.com/read/28734838/expression-purification-and-characterization-of-sll1981-protein-from-cyanobacterium-synechocystis-sp-pcc6803
#11
Xiaoqin Wang, Guofeng Lei, Xiaoyu Wu, Fei Wang, Chongde Lai, Zhimin Li
The sll1981 protein from cyanobacterium Synechocystis sp. PCC6803 had been reported to exhibit acetolactate synthase (ALS) and L-myo-inositol-1-phosphate synthase (MIPS) activities previously. Based on amino acids sequences alignment, sll1981 protein was postulated to function as α-ketoglutarate decarboxylase (α-KGD), which played important role in completing cyanobacterial tricarboxylic acid (TCA) cycle. However the detailed enzymatic kinetics of sll1981 as ALS, MIPS and α-KGD were not determined yet. In this study, the recombinant sll1981 protein was purified from supernatant of E...
July 19, 2017: Protein Expression and Purification
https://www.readbyqxmd.com/read/28734354/determination-of-aqueous-antibiotic-solutions-using-sers-nanogratings
#12
Koh Yiin Hong, Carlos Diego Lima de Albuquerque, Ronei J Poppi, Alexandre G Brolo
The emergence of antibiotics and their active metabolites in aquatic ecosystem has motivated the development of sensitive and reliable sensors to monitor traces of antibiotics and metabolites in drinking water sources (i.e. surface water). The surface enhanced Raman scattering (SERS) technique, which is widely recognized as a high sensitivity method for molecular vibrational detection, is potentially a powerful tool for trace environmental contamination analysis. The main goal of this work is to demonstrate pharmaceutical and metabolite multiplexing detection using the SERS approach...
August 22, 2017: Analytica Chimica Acta
https://www.readbyqxmd.com/read/28734097/first-report-of-ser653-asn-mutation-endowing-high-level-resistance-to-imazamox-in-downy-brome-bromus-tectorum-l
#13
Vipan Kumar, Prashant Jha
BACKGROUND: Bromus tectorum L. is one of the most troublesome grass weed species in cropland and non-cropland areas of the northwestern United States. In summer 2016, a B. tectroum accession (R) that survived imazamox at the field-use rate (44 g ha(-1) ) in an imidazolinone-tolerant (IMI-tolerant or Clearfield(™) ) winter wheat field was collected near Hammond, Carter County, MT, USA. The aim of this study was to determine the resistance profile of the B. tectroum R accession to imazamox and other ALS inhibitors, and investigate the mechanism of resistance to imazamox...
July 22, 2017: Pest Management Science
https://www.readbyqxmd.com/read/28732762/effect-of-class-ii-hdac-inhibition-on-glutamate-transporter-expression-and-survival-in-sod1-als-mice
#14
Andrea Lapucci, Leonardo Cavone, Daniela Buonvicino, Roberta Felici, Elisabetta Gerace, Clemens Zwergel, Sergio Valente, Antonello Mai, Alberto Chiarugi
Transcriptional deregulation emerges as a key pathogenetic mechanism in ALS pathogenesis, and non-class-specific histone deacetylase (HDACs) inhibitors proved of therapeutic efficacy in preclinical models of ALS. When tested in patients, however, these drugs failed, probably because of a lack of selectivity toward pathogenetic HDACs. Here, we studied the effects of MC1568, an inhibitor of Class-II HDACs which have been reported to contribute to ALS pathogenesis. We focused on transcriptional regulation of glutamate transporter EAAT2, whose reduced expression may contribute to motor neuron degeneration in ALS...
July 18, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/28729824/mouse-models-of-c9orf72-hexanucleotide-repeat-expansion-in-amyotrophic-lateral-sclerosis-frontotemporal-dementia
#15
REVIEW
Ranjan Batra, Chris W Lee
The presence of hexanucleotide repeat expansion (HRE) in the first intron of the human C9orf72 gene is the most common genetic cause underlying both familial amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Studies aimed at elucidating the pathogenic mechanisms associated of C9orf72 FTD and ALS (C9FTD/ALS) have focused on the hypothesis of RNA and protein toxic gain-of-function models, including formation of nuclear RNA foci containing GGGGCC (G4C2) HRE, inclusions containing dipeptide repeat proteins through a non-canonical repeat associated non-ATG (RAN) translation mechanism, and on loss-of-function of the C9orf72 protein...
2017: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/28728441/ability-of-pulmonary-function-decline-to-predict-death-in-amyotrophic-lateral-sclerosis-patients
#16
Irina Enache, Cristina Pistea, Marie Fleury, Mickael Schaeffer, Monique Oswald-Mammosser, Andoni Echaniz-Laguna, Christine Tranchant, Nicolas Meyer, Anne Charloux
OBJECTIVES: Objectives were to evaluate the relative risk of death associated with lung function decline in patients with amyotrophic lateral sclerosis (ALS), and to examine the ability of ALS patients to perform volitional pulmonary function tests (PFTs). METHODS: The PFTs of 256 consecutive patients referred to the Strasbourg University Hospital ALS Centre over an eight-year period were reviewed. Slow vital capacity (VC), maximal inspiratory and expiratory pressures (MIP, MEP), sniff nasal inspiratory pressure (SNIP), and peak cough flow (PCF) were performed at diagnosis and then every four months...
July 20, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28727995/physician-perceptions-about-living-organ-donation-in-patients-with-amyotrophic-lateral-sclerosis
#17
S Ansari, M B Bromberg, S B Gibson
OBJECTIVES: Patients with Amyotrophic Lateral Sclerosis (ALS) have expressed desire to become living organ donors but are unable to do so with current organ donation policies. Our objective is to assess ALS patient's interest in organ donation, and perceived concerns of this practice by ALS neurologists. PATIENTS AND METHODS: An electronic survey was administered to ALS neurologists across the United States regarding living organ donation in ALS patients prior to respiratory failure...
July 5, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28727554/a-human-humanoid-interaction-through-the-use-of-bci-for-locked-in-als-patients-using-neuro-biological-feedback-fusion
#18
Rosario Sorbello, Salvatore Tramonte, Marcello Giardina, Vincenzo La Bella, Rossella Spataro, Brendan Allison, Christoph Guger, Antonio Chella
This paper illustrates a new architecture for a human-humanoid interaction based on EEG-Brain Computer Interface (EEG-BCI) for patients affected by locked-in syndrome caused by Amyotrophic Lateral Sclerosis (ALS). The proposed architecture is able to recognise users' mental state accordingly to the biofeedback factor Bf , based on users' Attention, Intention and Focus, that is used to elicit a robot to perform customised behaviours. Experiments have been conducted with a population of 8 subjects: 4 ALS patients in a near Locked-in status with normal ocular movement and 4 healthy control subjects enrolled for age, education and computer expertise...
July 18, 2017: IEEE Transactions on Neural Systems and Rehabilitation Engineering
https://www.readbyqxmd.com/read/28725179/fine-tuning-er-stress-signal-transducers-to-treat-amyotrophic-lateral-sclerosis
#19
Danilo B Medinas, Jose V González, Paulina Falcon, Claudio Hetz
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by the progressive loss of motoneurons and paralysis. The mechanisms underlying neuronal degeneration in ALS are starting to be elucidated, highlighting disturbances in motoneuron proteostasis. Endoplasmic reticulum (ER) stress has emerged as an early pathogenic event underlying motoneuron vulnerability and denervation in ALS. Maintenance of ER proteostasis is controlled by a dynamic signaling network known as the unfolded protein response (UPR)...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28723387/sigma-1-receptor-activation-modifies-intracellular-calcium-exchange-in-the-g93a-hsod1-als-model
#20
Vedrana Tadić, Ayse Malci, Nadine Goldhammer, Beatrice Stubendorff, Saikata Sengupta, Tino Prell, Silke Keiner, Jingyu Liu, Madlen Guenther, Christiane Frahm, Otto W Witte, Julian Grosskreutz
Aberrations in intracellular calcium (Ca(2+)) have been well established within amyotrophic lateral sclerosis (ALS), a severe motor neuron disease. Intracellular Ca(2+) concentration is controlled in part through the endoplasmic reticulum (ER) mitochondria Ca(2+) cycle (ERMCC). The ER supplies Ca(2+) to the mitochondria at close contacts between the two organelles, i.e. the mitochondria-associated ER membranes (MAMs). The Sigma 1 receptor (Sig1R) is enriched at MAMs, where it acts as an inter-organelle signaling modulator...
July 16, 2017: Neuroscience
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