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https://www.readbyqxmd.com/read/28550440/csf-concentrations-of-adipsin-and-adiponectin-in-patients-with-amyotrophic-lateral-sclerosis
#1
Héctor R Martínez, César E Escamilla-Ocañas, Carlos R Camara-Lemarroy, María T González-Garza, Juan M Tenorio-Pedraza, Martín Hernández-Torre
Amyotrophic lateral sclerosis (ALS) is described as a neurodegenerative disorder. However, neuroinflammation and chemokine expression are prominent pathological finding at sites of injury. Adipsin and adiponectin are molecules that are implicated in the pathogenesis of neurodegenerative and neuroimmune disorders. Adipsin and adiponectin concentrations were determined in the CSF of ALS patients and controls and the relationship of these chemokines with clinical severity and disease duration in ALS was determined...
May 26, 2017: Acta Neurologica Belgica
https://www.readbyqxmd.com/read/28550099/viral-delivery-of-c9orf72-hexanucleotide-repeat-expansions-in-mice-lead-to-repeat-length-dependent-neuropathology-and-behavioral-deficits
#2
Saul Herranz-Martin, Jayanth Chandran, Katherine Lewis, Padraig Mulcahy, Adrian Higginbottom, Callum Walker, Isabel Martinez-Pena Y Valenzuela, Ross A Jones, Ian Coldicott, Tommaso Iannitti, Mohammed Akaaboune, Sherif F El-Khamisy, Thomas H Gillingwater, Pamela J Shaw, Mimoun Azzouz
Intronic GGGGCC repeat expansions in C9orf72 are the most common genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Two major pathologies stemming from the hexanucleotide RNA expansions (HREs) have been identified in postmortem tissue: intracellular RNA foci and repeat-associated non-ATG dependent (RAN) dipeptides, though it is unclear how these and other hallmarks of disease contribute to the pathophysiology of neuronal injury. Here we generated two novel lines of mice that overexpress either 10 pure or 102 interrupted G4C2 repeats mediated by adeno-associated virus (AAV) and characterized relevant pathology and disease-related behavioral phenotypes...
May 26, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28549443/quantitative-analysis-of-cryptic-splicing-associated-with-tdp-43-depletion
#3
Jack Humphrey, Warren Emmett, Pietro Fratta, Adrian M Isaacs, Vincent Plagnol
BACKGROUND: Reliable exon recognition is key to the splicing of pre-mRNAs into mature mRNAs. TDP-43 is an RNA-binding protein whose nuclear loss and cytoplasmic aggregation are a hallmark pathology in amyotrophic lateral sclerosis and frontotemporal dementia (ALS/FTD). TDP-43 depletion causes the aberrant inclusion of cryptic exons into a range of transcripts, but their extent, relevance to disease pathogenesis and whether they are caused by other RNA-binding proteins implicated in ALS/FTD are unknown...
May 26, 2017: BMC Medical Genomics
https://www.readbyqxmd.com/read/28545479/endoplasmic-reticulum-stress-and-inflammation-in-the-central-nervous-system
#4
REVIEW
Neil T Sprenkle, Savannah G Sims, Cristina L Sánchez, Gordon P Meares
Persistent endoplasmic reticulum (ER) stress is thought to drive the pathology of many chronic disorders due to its potential to elicit aberrant inflammatory signaling and facilitate cell death. In neurodegenerative diseases, the accumulation of misfolded proteins and concomitant induction of ER stress in neurons contributes to neuronal dysfunction. In addition, ER stress responses induced in the surrounding neuroglia may promote disease progression by coordinating damaging inflammatory responses, which help fuel a neurotoxic milieu...
May 25, 2017: Molecular Neurodegeneration
https://www.readbyqxmd.com/read/28544463/axonal-charcot-marie-tooth-neuropathy-concurrent-with-distal-and-proximal-weakness-by-translational-elongation-of-the-3-utr-in-nefh
#5
Da Eun Nam, Sung-Chul Jung, Da Hye Yoo, Sun Seong Choi, Sung-Yum Seo, Gwang Hoon Kim, Song-Ja Kim, Soo Hyun Nam, Byung-Ok Choi, Ki Wha Chung
Mutations in the NEFH gene encoding the heavy neurofilament protein are usually associated with neuronal damage and susceptibility to amyotrophic lateral sclerosis (ALS). Recently, frameshift variants in NEFH (p.Asp1004Glnfs*58 and p.Pro1008Alafs*56) have been reported to be the underlying cause of axonal Charcot-Marie-Tooth disease type 2CC (CMT2CC). The frameshift mutation resulted in a stop loss and translation of a cryptic amyloidogenic element (CAE) encoded by the 3' UTR. This study also identified a de novo c...
May 24, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28544227/mindfulness-depression-and-quality-of-life-in-amyotrophic-lateral-sclerosis
#6
EDITORIAL
J S Lou
No abstract text is available yet for this article.
May 23, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28543166/chronic-emgs-in-treadmill-running-sod1-mice-reveal-early-changes-in-muscle-activation
#7
K A Quinlan, E Kajtaz, J D Ciolino, R D Imhoff-Manuel, M C Tresch, C J Heckman, V M Tysseling
To improve our understanding of early disease mechanisms and find reliable biomarkers of amyotrophic lateral sclerosis (ALS), a progressive neurodegenerative disease, we measured electromyogram (EMG) activity in hind limb muscles of SOD1G93A mice. In contrast to clinical diagnostic measures using EMGs, which are performed on quiescent patients, we monitored activity during treadmill running in order to detect presymptomatic changes in motor patterning. Chronic EMG electrodes were implanted into vastus lateralis (VL), biceps femoris posterior (BFP), lateral gastrocnemius (LG), and tibialis anterior (TA) in mice from postnatal day (P) 55-100, and results were assessed using linear mixed models...
May 24, 2017: Journal of Physiology
https://www.readbyqxmd.com/read/28542233/is-survival-improved-by-the-use-of-niv-and-peg-in-amyotrophic-lateral-sclerosis-als-a-post-mortem-study-of-80-als-patients
#8
Christian Burkhardt, Christoph Neuwirth, Andreas Sommacal, Peter M Andersen, Markus Weber
BACKGROUND: Non-invasive ventilation (NIV) and percutaneous gastrostomy (PEG) are guideline-recommended interventions for symptom management in amyotrophic lateral sclerosis (ALS). Their effect on survival is controversial and the impact on causes of death is unknown. OBJECTIVE: To investigate the effect of NIV and PEG on survival and causes of death in ALS patients. METHODS: Eighty deceased ALS patients underwent a complete post mortem analysis for causes of death between 2003 and 2015...
2017: PloS One
https://www.readbyqxmd.com/read/28541074/purpose-in-life-in-als-patient-caregiver-dyads-a-multilevel-longitudinal-analysis
#9
Natasha E Garcia, Jennifer N Morey, Edward J Kasarskis, Suzanne C Segerstrom
OBJECTIVE: Eudaemonic positive psychological health (PPH), such as purpose in life (PIL), may be maintained more than hedonic PPH, such as quality of life (QOL), for patients with amyotrophic lateral sclerosis (ALS) and their caregivers across the disease course. Furthermore, patients' and caregivers' PPH may impact one another. The present study examined (a) PIL and QOL variance structures; (b) PIL and QOL trajectories from diagnosis, approaching death, with disease severity; and (c) between-dyad and within-dyad relationships for PIL and QOL in patients with ALS and their caregivers...
May 25, 2017: Health Psychology: Official Journal of the Division of Health Psychology, American Psychological Association
https://www.readbyqxmd.com/read/28540663/bmaa-and-neurodegenerative-illness
#10
Paul Alan Cox, Richard M Kostrzewa, Gilles J Guillemin
The cyanobacterial toxin β-N-methylamino-L-alanine (BMAA) now appears to be a cause of Guamanian amyotrophic lateral sclerosis/parkinsonism dementia complex (ALS/PDC). Its production by cyanobacteria throughout the world combined with multiple mechanisms of BMAA neurotoxicity, particularly to vulnerable subpopulations of motor neurons, has significantly increased interest in investigating exposure to this non-protein amino acid as a possible risk factor for other forms of neurodegenerative illness. We here provide a brief overview of BMAA studies and provide an introduction to this collection of scientific manuscripts in this special issue on BMAA...
May 24, 2017: Neurotoxicity Research
https://www.readbyqxmd.com/read/28539884/the-analysis-of-two-bdnf-polymorphisms-g196a-c270t-in-chinese-sporadic-amyotrophic-lateral-sclerosis
#11
Lianping Xu, Danyang Tian, Jiao Li, Lu Chen, Lu Tang, Dongsheng Fan
Amyotrophic lateral sclerosis (ALS) is an ethnically heterogeneous motor neuron disease that results from the selective death of motor neurons in the brain and spinal cord. Brain-derived neurotrophic factor (BDNF) is widely distributed across the central and peripheral nervous systems and plays neurotrophic and other physiological roles in various brain regions. Alterations of neurotrophin availability have been proposed as a pathogenic mechanism underlying ALS neurodegeneration. Several genetic studies have shown a significant association between schizophrenia, Alzheimer's disease, and Parkinson's disease and certain BDNF polymorphisms, specifically G196A (rs6265) and C270T (rs56164415)...
2017: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/28539871/protein-quality-control-and-the-amyotrophic-lateral-sclerosis-frontotemporal-dementia-continuum
#12
Hamideh Shahheydari, Audrey Ragagnin, Adam K Walker, Reka P Toth, Marta Vidal, Cyril J Jagaraj, Emma R Perri, Anna Konopka, Jessica M Sultana, Julie D Atkin
Protein homeostasis, or proteostasis, has an important regulatory role in cellular function. Protein quality control mechanisms, including protein folding and protein degradation processes, have a crucial function in post-mitotic neurons. Cellular protein quality control relies on multiple strategies, including molecular chaperones, autophagy, the ubiquitin proteasome system, endoplasmic reticulum (ER)-associated degradation (ERAD) and the formation of stress granules (SGs), to regulate proteostasis. Neurodegenerative diseases are characterized by the presence of misfolded protein aggregates, implying that protein quality control mechanisms are dysfunctional in these conditions...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28539831/aldehyde-dehydrogenases-1a2-expression-and-distribution-are-potentially-associated-with-neuron-death-in-spinal-cord-of-tg-sod1-g93a-1gur-mice
#13
Huiting Liang, Chengsi Wu, Youqing Deng, Lei Zhu, Jie Zhang, Weiming Gan, Chunyan Tang, Renshi Xu
The pathogenesis of amyotrophic lateral sclerosis (ALS) has not been unclear yet, it might be associated with the abnormal expression and distribution of certain proteins. Aldehyde dehydrogenases 1A2 (ALDH1A2) was thought to be one of potential candidates. Therefore, in this study we observed and analyzed the alteration of the expression and distribution of ALDH1A2 in the spinal cord of wild-type (WT) and Tg(SOD1*G93A)1Gur mice. We compared the expression and distribution of ALDH1A2 in the different segments, anatomic regions and neural cells of spinal cord at the different stages of WT and Tg(SOD1*G93A)1Gur mice applied the methods of fluorescent immunohistochemistry and western blot...
2017: International Journal of Biological Sciences
https://www.readbyqxmd.com/read/28539470/the-src-c-abl-pathway-is-a-potential-therapeutic-target-in-amyotrophic-lateral-sclerosis
#14
Keiko Imamura, Yuishin Izumi, Akira Watanabe, Kayoko Tsukita, Knut Woltjen, Takuya Yamamoto, Akitsu Hotta, Takayuki Kondo, Shiho Kitaoka, Akira Ohta, Akito Tanaka, Dai Watanabe, Mitsuya Morita, Hiroshi Takuma, Akira Tamaoka, Tilo Kunath, Selina Wray, Hirokazu Furuya, Takumi Era, Kouki Makioka, Koichi Okamoto, Takao Fujisawa, Hideki Nishitoh, Kengo Homma, Hidenori Ichijo, Jean-Pierre Julien, Nanako Obata, Masato Hosokawa, Haruhiko Akiyama, Satoshi Kaneko, Takashi Ayaki, Hidefumi Ito, Ryuji Kaji, Ryosuke Takahashi, Shinya Yamanaka, Haruhisa Inoue
Amyotrophic lateral sclerosis (ALS), a fatal disease causing progressive loss of motor neurons, still has no effective treatment. We developed a phenotypic screen to repurpose existing drugs using ALS motor neuron survival as readout. Motor neurons were generated from induced pluripotent stem cells (iPSCs) derived from an ALS patient with a mutation in superoxide dismutase 1 (SOD1). Results of the screen showed that more than half of the hits targeted the Src/c-Abl signaling pathway. Src/c-Abl inhibitors increased survival of ALS iPSC-derived motor neurons in vitro...
May 24, 2017: Science Translational Medicine
https://www.readbyqxmd.com/read/28537509/maugeri-centre-for-telehealth-and-telecare-a-real-life-integrated-experience-in-chronic-patients
#15
Simonetta Scalvini, Palmira Bernocchi, Emanuela Zanelli, Laura Comini, Michele Vitacca
Management of chronic diseases in a progressively aging population is a major issue in western industrialized countries and telehealth is one way to ensure the continuity of care in chronic illness. We describe here our personal experience in a telehealth and telecare centre in Italy. Between January 2000 and December 2015, 1635 elderly patients (71% male) with one or more comorbidities have undergone a telehealth program tailored to their specific disease: chronic obstructive pulmonary disease (COPD)/chronic respiratory insufficiency; amyotrophic lateral sclerosis/neuromuscular diseases; chronic heart failure (CHF); post-stroke; and post-cardiac surgery patients discharged from hospital after an acute event...
January 1, 2017: Journal of Telemedicine and Telecare
https://www.readbyqxmd.com/read/28537420/nitration-and-glycation-turn-mature-ngf-into-a-toxic-factor-for-motor-neurons-a-role-for-p75-sup-ntr-sup-and-rage-signaling-in-als
#16
Mi Jin Kim, Marcelo R Vargas, Benjamin A Harlan, Kelby M Killoy, Lauren Ball, Susana Comte-Walters, Monika Gooz, Yasuhiko Yamamoto, Joseph S Beckman, Luis Barbeito, Mariana Pehar
Glycating stress can occur together with oxidative stress during neurodegeneration and contribute to the pathogenic mechanism. Nerve growth factor (NGF) accumulates in several neurodegenerative diseases. Besides promoting survival, NGF can paradoxically induce cell death by signaling through the p75 neurotrophin receptor (p75<sup>NTR</sup>). The ability of NGF to induce cell death is increased by nitration of its tyrosine residues under conditions associated with increased peroxynitrite formation...
May 24, 2017: Antioxidants & Redox Signaling
https://www.readbyqxmd.com/read/28536907/drug-use-in-older-adults-with-amyotrophic-lateral-sclerosis-near-the-end-of-life
#17
Giulia Grande, Lucas Morin, Davide Liborio Vetrano, Johan Fastbom, Kristina Johnell
BACKGROUND: Amyotrophic lateral sclerosis (ALS), with its certain prognosis and swift progression, raises concerns regarding the adequacy of pharmacological treatment, including the risk-benefit profiles of prescribed drugs. OBJECTIVE: Our objective was to evaluate the use of prescription drugs over the course of the last year of life in older adults with ALS. METHODS: We conducted a nationwide retrospective cohort study of older adults who died with ALS in Sweden between 2007 and 2013...
May 23, 2017: Drugs & Aging
https://www.readbyqxmd.com/read/28536362/functionalisation-of-polyvinylpyrrolidone-on-gold-nanoparticles-enhances-its-anti-amyloidogenic-propensity-towards-hen-egg-white-lysozyme
#18
Tulika Das, Vidyalatha Kolli, Srijeeb Karmakar, Nandini Sarkar
Protein amyloids are characterized by aggregates that usually consist of fibres containing misfolded proteins and having a cross β-sheet conformation. These aggregates can eventually lead to several degenerative diseases like Alzheimer's disease, amyotrophic lateral sclerosis (ALS), Huntington's disease and Parkinson's disease. The present study describes the effect of chemically synthesized polyvinylpyrrolidone (PVP)-conjugated gold nanoparticles (PVP-AuNps) on hen egg white lysozyme (HEWL) amyloids. The synthesized nanoparticles have been characterized using various biophysical techniques like Ultraviolet-Visible (UV-Vis) Spectroscopy, Transmission electron microscopy (TEM), X-ray diffraction (XRD) analysis, dynamic light scattering (DLS), zeta-potential measurement and Fourier transform infrared spectroscopy (FTIR)...
May 3, 2017: Biomedicines
https://www.readbyqxmd.com/read/28533943/etiology-and-treatment-of-amyotrophic-lateral-sclerosis
#19
Hernando Rafael, Juan Oscar David, Antonio Santiago Vilca
BACKGROUND: To date all researchers conclude that the etiology of Amyotrophic lateral sclerosis (ALS) is not known. On the contrary, since August 2009, we believe that disease is of ischemic origin in the anterior surface of the medulla oblongata. MATERIAL AND METHOD: We present our surgical experience into 45 patients with ALS (bulbar form in 36 cases and spinal form in 9). Preoperative MRI scans revealed microinfarcts in the medulla oblongata and/or cervical cord...
2017: American Journal of Neurodegenerative Disease
https://www.readbyqxmd.com/read/28533621/correlations-between-slow-rate-repetitive-nerve-stimulation-and-characteristics-associated-with-amyotrophic-lateral-sclerosis-in-chinese-patients
#20
Yan Wang, Zheman Xiao, Hong Chu, Jingjing Liang, Xu Wu, Hongjuan Dong, Yang Yan, Zuneng Lu
[Purpose] To clarify the features associated with decrements in compound muscle action potentials (CMAP) during slow-rate repetitive nerve stimulation (RNS) of muscles involved in amyotrophic lateral sclerosis (ALS) in mainland China. [Subjects and Methods] A retrospective study of decremental responses to slow-rate RNS was performed to compare patients with ALS to those with myasthenia gravis (MG). [Results] A significant decrement (>5%) was observed in at least one muscle in 54% of ALS patients. The trapezius muscle was the most commonly affected (67%)...
April 2017: Journal of Physical Therapy Science
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