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movement and neurodegeneration

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https://www.readbyqxmd.com/read/28931001/an-automated-rapid-iterative-negative-geotaxis-assay-for-analyzing-adult-climbing-behavior-in-a-drosophila-model-of-neurodegeneration
#1
Wenze Cao, Li Song, Jingjing Cheng, Na Yi, Luyi Cai, Fu-de Huang, Margaret Ho
Neurodegenerative diseases are frequently associated with a progressive loss of movement ability, reduced life span, and age-dependent neurodegeneration. To understand the mechanism of these cellular events, and their causal relationships with each other, Drosophila melanogaster, with its sophisticated genetic tools and diverse behavioral features, are used as disease models for assessing neurodegenerative phenotypes. Here we describe a high-throughput method to analyze Drosophila adult negative geotaxis behavior, as an indication for possible motor defects associated with neurodegeneration...
September 12, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/28905504/is-essential-tremor-a-single-entity
#2
Franziska Hopfner, Günther Deuschl
Essential tremor (ET) is a frequent movement disorder. A new tremor classification has subdivided ET, into the classical form with bilateral action tremor of the hands with or without involvement of further tremor locations and without any other explaining signs or symptoms for the tremor and into 'ET plus' which comes additionally with further neurologic signs of unknown origin. This will provide a better foundation for subclassifying the condition. The immediate cause of ET is a preformed oscillating network within the central nervous system as revealed with electrophysiologic methods...
September 14, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28904337/toxoplasma-modulates-signature-pathways-of-human-epilepsy-neurodegeneration-cancer
#3
Huân M Ngô, Ying Zhou, Hernan Lorenzi, Kai Wang, Taek-Kyun Kim, Yong Zhou, Kamal El Bissati, Ernest Mui, Laura Fraczek, Seesandra V Rajagopala, Craig W Roberts, Fiona L Henriquez, Alexandre Montpetit, Jenefer M Blackwell, Sarra E Jamieson, Kelsey Wheeler, Ian J Begeman, Carlos Naranjo-Galvis, Ney Alliey-Rodriguez, Roderick G Davis, Liliana Soroceanu, Charles Cobbs, Dennis A Steindler, Kenneth Boyer, A Gwendolyn Noble, Charles N Swisher, Peter T Heydemann, Peter Rabiah, Shawn Withers, Patricia Soteropoulos, Leroy Hood, Rima McLeod
One third of humans are infected lifelong with the brain-dwelling, protozoan parasite, Toxoplasma gondii. Approximately fifteen million of these have congenital toxoplasmosis. Although neurobehavioral disease is associated with seropositivity, causality is unproven. To better understand what this parasite does to human brains, we performed a comprehensive systems analysis of the infected brain: We identified susceptibility genes for congenital toxoplasmosis in our cohort of infected humans and found these genes are expressed in human brain...
September 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28902452/mgrn1-mediated-ubiquitination-of-%C3%AE-tubulin-regulates-microtubule-dynamics-and-intracellular-transport
#4
Rukmini Mukherjee, Priyanka Majumder, Oishee Chakrabarti
MGRN1 mediated ubiquitination of α-tubulin regulates microtubule stability and mitotic spindle positioning in mitotic cells. This study elucidates the effect of MGRN1 mediated ubiquitination of α-tubulin in interphase cells. Here, we show that MGRN1 mediated ubiquitination regulates dynamics of EB1 labelled plus ends of microtubules. Intracellular transport of mitochondria and endosomes are affected in cultured cells where functional MGRN1 is depleted. Defects in microtubule-dependent organellar transport are evident in cells where noncanonical K6 mediated ubiquitination of α-tubulin by MGRN1 is compromised...
September 13, 2017: Traffic
https://www.readbyqxmd.com/read/28861893/glitazone-use-associated-with-reduced-risk-of-parkinson-s-disease
#5
Brage Brakedal, Irene Flønes, Simone F Reiter, Øivind Torkildsen, Christian Dölle, Jörg Assmus, Kristoffer Haugarvoll, Charalampos Tzoulis
BACKGROUND: Whether antidiabetic glitazone drugs protect against Parkinson's disease remains controversial. Although a single clinical trial showed no evidence of disease modulation, retrospective studies suggest that a disease-preventing effect may be plausible. The objective of this study was to examine if the use of glitazone drugs is associated with a lower incidence of PD among diabetic patients. METHODS: We compared the incidence of PD between individuals with diabetes who used glitazones, with or without metformin, and individuals using only metformin in the Norwegian Prescription Database...
September 1, 2017: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/28845923/clinical-rating-scale-for-pantothenate-kinase-associated-neurodegeneration-a-pilot-study
#6
Alejandra Darling, Cristina Tello, María Josep Martí, Cristina Garrido, Sergio Aguilera-Albesa, Miguel Tomás Vila, Itziar Gastón, Marcos Madruga, Luis González Gutiérrez, Julio Ramos Lizana, Montserrat Pujol, Tania Gavilán Iglesias, Kylee Tustin, Jean Pierre Lin, Giovanna Zorzi, Nardo Nardocci, Loreto Martorell, Gustavo Lorenzo Sanz, Fuencisla Gutiérrez, Pedro J García, Lidia Vela, Carlos Hernández Lahoz, Juan Darío Ortigoza Escobar, Laura Martí Sánchez, Fradique Moreira, Miguel Coelho, Leonor Correia Guedes, Ana Castro Caldas, Joaquim Ferreira, Paula Pires, Cristina Costa, Paulo Rego, Marina Magalhães, María Stamelou, Daniel Cuadras Pallejà, Carmen Rodríguez-Blazquez, Pablo Martínez-Martín, Vincenzo Lupo, Leonidas Stefanis, Roser Pons, Carmen Espinós, Teresa Temudo, Belén Pérez Dueñas
BACKGROUND: Pantothenate kinase-associated neurodegeneration is a progressive neurological disorder occurring in both childhood and adulthood. The objective of this study was to design and pilot-test a disease-specific clinical rating scale for the assessment of patients with pantothenate kinase-associated neurodegeneration. METHODS: In this international cross-sectional study, patients were examined at the referral centers following a standardized protocol. The motor examination was filmed, allowing 3 independent specialists in movement disorders to analyze 28 patients for interrater reliability assessment...
August 28, 2017: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/28836304/impaired-microglia-process-dynamics-post-stroke-are-specific-to-sites-of-secondary-neurodegeneration
#7
Murielle G Kluge, Laura Kracht, Mahmoud Abdolhoseini, Lin Kooi Ong, Sarah J Johnson, Michael Nilsson, Frederick R Walker
Stroke induces tissue death both at the site of infarction and at secondary sites connected to the primary infarction. This latter process has been referred to as secondary neurodegeneration (SND). Using predominantly fixed tissue analyses, microglia have been implicated in regulating the initial response at both damage sites post-stroke. In this study, we used acute slice based multiphoton imaging, to investigate microglia dynamic process movement in mice 14 days after a photothrombotic stroke. We evaluated the baseline motility and process responses to locally induced laser damage in both the peri-infarct (PI) territory and the ipsilateral thalamus, a major site of post-stroke SND...
August 24, 2017: Glia
https://www.readbyqxmd.com/read/28821231/novel-mutations-in-pank2-and-pla2g6-genes-in-patients-with-neurodegenerative-disorders-two-case-reports
#8
Hassan Dastsooz, Hamid Nemati, Mohammad Ali Farazi Fard, Majid Fardaei, Mohammad Ali Faghihi
BACKGROUND: Neurodegeneration with brain iron accumulation (NBIA) is a genetically heterogeneous group of disorders associated with progressive impairment of movement, vision, and cognition. The disease is initially diagnosed on the basis of changes in brain magnetic resonance imaging which indicate an abnormal brain iron accumulation in the basal ganglia. However, the diagnosis of specific types should be based on both clinical findings and molecular genetic testing for genes associated with different types of NBIA, including PANK2, PLA2G6, C19orf12, FA2H, ATP13A2, WDR45, COASY, FTL, CP, and DCAF17...
August 18, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28819888/paraquat-induced-movement-disorder-in-relation-to-oxidative-stress-mediated-neurodegeneration-in-the-brain-of-drosophila-melanogaster
#9
S Niveditha, S R Ramesh, T Shivanandappa
Exposure to pesticides like paraquat (PQ) is considered as a risk factor for Parkinson's disease (PD). PQ has been shown to induce PD-like phenotype in experimental animals. Drosophila, a valuable laboratory model organism, is widely used to study neurodegenerative disorders including PD. The acute (single dose) PQ model of PD in Drosophila is associated with high mortality as well as reversibility of locomotor deficits and, therefore, does not replicate the disease phenotype. We have investigated the relevance of the acute and multiple (sublethal) dose of PQ to induce PD-like symptoms in Drosophila and shown that multiple-dose of PQ induces irreversible locomotor impairment without significant mortality...
August 18, 2017: Neurochemical Research
https://www.readbyqxmd.com/read/28790966/randomized-controlled-study-of-a-remote-flipped-classroom-neuro-otology-curriculum
#10
Frederick Robert Carrick, Mahera Abdulrahman, Ahmed Hankir, Maksim Zayaruzny, Kinda Najem, Palita Lungchukiet, Roger A Edwards
CONTEXT: Medical Education can be delivered in the traditional classroom or via novel technology including an online classroom. OBJECTIVE: To test the hypothesis that learning in an online classroom would result in similar outcomes as learning in the traditional classroom when using a flipped classroom pedagogy. DESIGN: Randomized controlled trial. A total of 274 subjects enrolled in a Neuro-otology training program for non-Neuro-otologists of 25 h held over a 3-day period...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28769866/eye-control-deficits-coupled-to-hand-control-deficits-eye-hand-incoordination-in-chronic-cerebral-injury
#11
John-Ross Rizzo, James K Fung, Maryam Hosseini, Azadeh Shafieesabet, Edmond Ahdoot, Rosa M Pasculli, Janet C Rucker, Preeti Raghavan, Michael S Landy, Todd E Hudson
It is widely accepted that cerebral pathology can impair ocular motor and manual motor control. This is true in indolent and chronic processes, such as neurodegeneration and in acute processes such as stroke or those secondary to neurotrauma. More recently, it has been suggested that disruptions in these control systems are useful markers for prognostication and longitudinal monitoring. The utility of examining the relationship or the coupling between these systems has yet to be determined. We measured eye and hand-movement control in chronic, middle cerebral artery stroke, relative to healthy controls, in saccade-to-reach paradigms to assess eye-hand coordination...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28681623/psychiatric-comorbidities-in-movement-disorders
#12
Adán Miguel-Puga, Gabriel Villafuerte, Oscar Arias-Carrión
Psychiatric comorbidities are common in movement disorders. This review provides a practical approach to help clinicians to recognize psychiatric disorders in the most frequent movement disorders. However, the extent of neurodegeneration, as well as the impact of medications with considerable CNS effects, influences the diverse psychiatric presentations that, in turn, are influenced by the stress of living with a movement disorder. Depression, anxiety, and psychosis are the most common psychiatric comorbidities in movement disorders and of the medications used to treat the motor disturbances...
July 6, 2017: International Review of Psychiatry
https://www.readbyqxmd.com/read/28674979/huntington-s-disease-pathogenic-mechanisms-and-therapeutic-targets
#13
Dean J Wright, Thibault Renoir, Laura J Gray, Anthony J Hannan
Huntington's disease (HD) is a tandem repeat disorder involving neurodegeneration and a complex combination of symptoms. These include psychiatric symptoms, cognitive deficits culminating in dementia, and the movement disorder epitomised by motor signs such as chorea. HD is caused by a CAG repeat expansion encoding an extended tract of the amino acid glutamine in the huntingtin protein. This polyglutamine expansion appears to induce a 'change of function', possibly a 'gain of function', in the huntingtin protein, which leads to various molecular and cellular cascades of pathogenesis...
2017: Advances in Neurobiology
https://www.readbyqxmd.com/read/28672841/clinicopathological-phenotype-and-genetics-of-x-linked-dystonia-parkinsonism-xdp-dyt3-lubag
#14
REVIEW
Toshitaka Kawarai, Ryoma Morigaki, Ryuji Kaji, Satoshi Goto
X-linked dystonia-parkinsonism (XDP; OMIM314250), also referred to as DYT3 dystonia or "Lubag" disease, was first described as an endemic disease in the Philippine island of Panay. XDP is an adult-onset movement disorder characterized by progressive and severe dystonia followed by overt parkinsonism in the later years of life. Among the primary monogenic dystonias, XDP has been identified as a transcriptional dysregulation syndrome with impaired expression of the TAF1 (TATA box-binding protein associated factor 1) gene, which is a critical component of the cellular transcription machinery...
June 26, 2017: Brain Sciences
https://www.readbyqxmd.com/read/28664402/reduced-proliferation-of-oligodendrocyte-progenitor-cells-in-the-postnatal-brain-of-dystonia-musculorum-mice
#15
M Ibrahim Hossain, Masao Horie, Hirohide Takebayashi
Dystonia musculorum (dt) mice show sensory neurodegeneration and movement disorder, such as dystonia and cerebellar ataxia. The causative gene Dystonin (Dst) encodes a cytoskeleton linker protein. Although sensory neurodegeneration has been well studied, glial cell responses in the central nervous system (CNS) are poorly understood. Here, we investigated cell proliferation in the CNS of Dst (Gt) homozygous mice using newly generated in situ hybridization (ISH) probes-Ki-67 and proliferating cell nuclear antigen (PCNA) probes-both of which effectively detect proliferating cells...
June 29, 2017: Neurochemical Research
https://www.readbyqxmd.com/read/28649609/insomnia-and-somnolence-in-idiopathic-rbd-a-prospective-cohort-study
#16
Ronald B Postuma, Jean-François Gagnon, Amelie Pelletier, Jacques Y Montplaisir
Although some sleep disorders are markers of prodromal Parkinson's disease and dementia with Lewy bodies, it is unclear whether insomnia and somnolence can predict disease. We assessed a large cohort of patients with idiopathic rapid eye movement sleep behavior disorder and age/sex matched controls, comparing the Epworth sleepiness scale, the Insomnia Severity Index, the Pittsburgh Sleep Quality Index, and polysomnographic variables. In those with repeated scales, we assessed change over time. Finally, we assessed whether sleep abnormalities predicted defined neurodegenerative disease...
2017: NPJ Parkinson's Disease
https://www.readbyqxmd.com/read/28643035/clinical-and-imaging-presentation-of-a%C3%A2-patient-with-beta-propeller-protein-associated-neurodegeneration-a%C3%A2-rare-and-sporadic-form-of-neurodegeneration-with-brain-iron-accumulation-nbia
#17
Elke Hattingen, Nikolaus Handke, Kirsten Cremer, Sabine Hoffjan, Guido Matthias Kukuk
Neurodegeneration with brain iron accumulation (NBIA) is a heterogeneous group of inherited neurologic disorders with iron accumulation in the basal ganglia, which share magnetic resonance (MR) imaging characteristics, histopathologic and clinical features. According to the affected basal nuclei, clinical features include extrapyramidal movement disorders and varying degrees of intellectual disability status. The most common NBIA subtype is caused by pathogenic variants in PANK2. The hallmark of MR imaging in patients with PANK2 mutations is an eye-of-the-tiger sign in the globus pallidus...
June 22, 2017: Clinical Neuroradiology
https://www.readbyqxmd.com/read/28619181/multifactorial-sleep-disturbance-in-parkinson-s-disease
#18
REVIEW
J Andrew Albers, Pratap Chand, A Michael Anch
Parkinson's disease (PD) is the second most common neurodegenerative disorder, ranking only behind Alzheimer's disease and affecting 2% of the population over the age of 65. Pathophysiologically, PD is characterized by selective degeneration of the dopaminergic neurons of the substantia nigra pars compacta (SNpc) and striatal dopamine depletion. Patients may also exhibit mild-to-severe degeneration of other central and peripheral nervous tissues. The most dramatic symptoms of the disease are profound dopamine-responsive motor disturbances, including bradykinesia, akinesia, rigidity, resting tremor, and postural instability...
July 2017: Sleep Medicine
https://www.readbyqxmd.com/read/28574926/hyposmia-symptoms-of-rapid-eye-movement-sleep-behavior-disorder-and-parkinsonian-motor-signs-suggest-prodromal-neurodegeneration-in-22q11-deletion-syndrome
#19
Ellen Buckley, Azeem Siddique, Alisdair McNeill
The 22q11 deletion syndrome (22q11DS) is one of the most common genomic disorders in humans. There is an increased risk of Parkinson's disease (PD) in individuals with 22q11DS. The characteristic motor features of PD begin when more than 50% of dopaminergic neurons in the substantia nigra have degenerated. Before this, there is a prodromal period, of up to 20 years, in which nonmotor features such as hyposmia, autonomic dysfunction, rapid eye movement sleep behavior disorder, and subtle motor dysfunction can occur...
August 2, 2017: Neuroreport
https://www.readbyqxmd.com/read/28564598/a-glio-protective-role-of-mir-263a-by-tuning-sensitivity-to-glutamate
#20
Sherry Shiying Aw, Isaac Kok Hwee Lim, Melissa Xue Mei Tang, Stephen Michael Cohen
Glutamate is a ubiquitous neurotransmitter, mediating information flow between neurons. Defects in the regulation of glutamatergic transmission can result in glutamate toxicity, which is associated with neurodegeneration. Interestingly, glutamate receptors are expressed in glia, but little is known about their function, and the effects of their misregulation, in these non-neuronal cells. Here, we report a glio-protective role for Drosophila mir-263a mediated by its regulation of glutamate receptor levels in glia...
May 30, 2017: Cell Reports
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