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movement and neurodegeneration

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https://www.readbyqxmd.com/read/29151169/parkinson-s-disease-experimental-models-and-reality
#1
REVIEW
Peizhou Jiang, Dennis W Dickson
Parkinson's disease (PD) is a chronic, progressive movement disorder of adults and the second most common neurodegenerative disease after Alzheimer's disease. Neuropathologic diagnosis of PD requires moderate-to-marked neuronal loss in the ventrolateral substantia nigra pars compacta and α-synuclein (αS) Lewy body pathology. Nigrostriatal dopaminergic neurodegeneration correlates with the Parkinsonian motor features, but involvement of other peripheral and central nervous system regions leads to a wide range of non-motor features...
November 18, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/29146937/tfeb-regulates-lysosomal-positioning-by-modulating-tmem55b-expression-and-jip4-recruitment-to-lysosomes
#2
Rose Willett, José A Martina, James P Zewe, Rachel Wills, Gerald R V Hammond, Rosa Puertollano
Lysosomal distribution is linked to the role of lysosomes in many cellular functions, including autophagosome degradation, cholesterol homeostasis, antigen presentation, and cell invasion. Alterations in lysosomal positioning contribute to different human pathologies, such as cancer, neurodegeneration, and lysosomal storage diseases. Here we report the identification of a novel mechanism of lysosomal trafficking regulation. We found that the lysosomal transmembrane protein TMEM55B recruits JIP4 to the lysosomal surface, inducing dynein-dependent transport of lysosomes toward the microtubules minus-end...
November 17, 2017: Nature Communications
https://www.readbyqxmd.com/read/29145189/rem-sleep-behavior-disorder
#3
Claudio L Bassetti, Panagiotis Bargiotas
Rapid eye movement sleep behavior disorder (RBD) is a brain disorder, characterized by the dream enactment during rapid eye movement (REM) sleep due to a lack of physiologic muscle atonia and increased muscle twitching. Schenk was the first to describe this disorder in 1986; however, few authors reported in the 1970-1980s loss of physiological muscle atonia combined with dream enactment in the course of brainstem disorders and as a consequence of alcoholism and antidepressant treatment. RBD affects less than 1% of the adult population, but can be found in up to 25-50% of neurodegenerative disorders including Parkinson's disease, multisystem atrophy, and dementia with Lewy body...
2018: Frontiers of Neurology and Neuroscience
https://www.readbyqxmd.com/read/29124790/neuropathology-of-genetic-synucleinopathies-with-parkinsonism-review-of-the-literature
#4
REVIEW
Susanne A Schneider, Roy N Alcalay
Clinical-pathological studies remain the gold-standard for the diagnosis of Parkinson's disease (PD). However, mounting data from genetic PD autopsies challenge the diagnosis of PD based on Lewy body pathology. Most of the confirmed genetic risks for PD show heterogenous neuropathology, even within kindreds, which may or may not include Lewy body pathology. We review the literature of genetic PD autopsies from cases with molecularly confirmed PD or parkinsonism and summarize main findings on SNCA (n = 25), Parkin (n = 20, 17 bi-allelic and 3 heterozygotes), PINK1 (n = 5, 1 bi-allelic and 4 heterozygotes), DJ-1 (n = 1), LRRK2 (n = 55), GBA (n = 10 Gaucher disease patients with parkinsonism), DNAJC13, GCH1, ATP13A2, PLA2G6 (n = 8 patients, 2 with PD), MPAN (n = 2), FBXO7, RAB39B, and ATXN2 (SCA2), as well as on 22q deletion syndrome (n = 3)...
November 2017: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/29081897/mitochondrial-respiration-in-intact-peripheral-blood-mononuclear-cells-and-sirtuin-3-activity-in-patients-with-movement-disorders
#5
Slawomir Michalak, Jolanta Florczak-Wyspiańska, Joanna Rybacka-Mossakowska, Wojciech Ambrosius, Krystyna Osztynowicz, Aleksandra Baszczuk, Wojciech Kozubski, Ewa Wysocka
OBJECTIVE: Mitochondrial dysfunction is considered a unifying pathophysiological explanation for movement disorders. Sirtuin 3 (SIRT3) exhibits deacetylase activity and antioxidant properties. The aim of the study was to analyze the mitochondrial respiration in peripheral blood mononuclear cells (PBMCs) and the SIRT3 activity in patients with movement disorders. METHODS: Mitochondrial respiration was analyzed in intact PBMCs using the ROUTINE, LEAK, electron transfer system (ETS), and residual oxygen consumption (ROX) protocol by means of high-resolution respirometry...
2017: Oxidative Medicine and Cellular Longevity
https://www.readbyqxmd.com/read/29076602/diffusion-tensor-imaging-marks-dopaminergic-and-serotonergic-lesions-in-the-parkinsonian-monkey
#6
Elise Météreau, Maude Beaudoin-Gobert, Sandra Duperrier, Stéphane Thobois, Léon Tremblay, Véronique Sgambato-Faure
BACKGROUND: Diffusion tensor imaging has received major interest to highlight markers of neurodegeneration in Parkinson's disease. Whether the alteration of diffusion parameters mostly depicts dopaminergic lesions or can also reveal serotonergic denervation remains a question. OBJECTIVES: The aim of this study was to determine the best diffusion tensor imaging markers of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) and 3,4-methylene-dioxy-methamphetamine (MDMA; also known as ecstasy) lesions in the nonhuman primate...
October 27, 2017: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/29072553/prospective-memory-impairment-in-idiopathic-rem-sleep-behavior-disorder
#7
Ondrej Bezdicek, Tomáš Nikolai, Jiří Nepožitek, Pavla Peřinová, David Kemlink, Pavel Dušek, Iva Příhodová, Simona Dostálová, Veronika Ibarburu, Jiří Trnka, Karel Kupka, Zuzana Mecková, Jiří Keller, Josef Vymazal, Evžen Růžička, Karel Šonka, Petr Dušek
OBJECTIVE: The aim of the present study was to investigate if prospective memory (PM) is impaired in idiopathic rapid eye movement (REM) sleep behavior disorder (iRBD). RBD is a parasomnia characterized by dream enactment and by REM sleep without muscle atonia. iRBD is considered as the initial stage of neurodegeneration with pathological storage of alpha-synuclein. METHOD: Sixty iRBD patients with polysomnography-confirmed RBD without parkinsonism and dementia and 30 demographically matched normal controls (NC) were enrolled in the present study...
October 26, 2017: Clinical Neuropsychologist
https://www.readbyqxmd.com/read/29061563/rescue-of-atxn3-neuronal-toxicity-in-c-elegans-by-chemical-modification-of-er-stress
#8
Yasmin Fardghassemi, Arnaud Tauffenberger, Sarah Gosselin, J Alex Parker
Background: Polyglutamine expansion diseases are a group of hereditary neurodegenerative disorders that develop when a CAG repeat in the causative genes are unstably expanded above a certain threshold. The expansion of trinucleotide CAG repeats cause hereditary adult-onset neurodegenerative disorders such as Huntington's disease, dentatorubral-pallidoluysian atrophy, spinobulbar muscular atrophy and multiple forms of spinocerebellar ataxia (SCA). The most common dominantly inherited spinocerebellar ataxia is the type 3 (SCA3) also known as Machado-Joseph disease (MJD), is an autosomal dominant, progressive neurological disorder...
October 23, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29053777/movement-disorders-with-neuronal-antibodies-syndromic-approach-genetic-parallels-and-pathophysiology
#9
Bettina Balint, Angela Vincent, Hans-Michael Meinck, Sarosh R Irani, Kailash P Bhatia
Movement disorders are a prominent and common feature in many autoantibody-associated neurological diseases, a group of potentially treatable conditions that can mimic infectious, metabolic or neurodegenerative disease. Certain movement disorders are likely to associate with certain autoantibodies; for example, the characteristic dyskinesias, chorea and dystonia associated with NMDAR antibodies, stiff person spectrum disorders with GAD, glycine receptor, amphiphysin or DPPX antibodies, specific paroxysmal dystonias with LGI1 antibodies, and cerebellar ataxia with various anti-neuronal antibodies...
September 25, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29031405/life-span-resolved-nanotoxicology-enables-identification-of-age-associated-neuromuscular-vulnerabilities-in-the-nematode-caenorhabditis-elegans
#10
Annette Piechulek, Anna von Mikecz
At present, the majority of investigations concerning nanotoxicology in the nematode C. elegans address short-term effects. While this approach allows for the identification of uptake pathways, exposition and acute toxicity, nanoparticle-organism interactions that manifest later in the adult life of C. elegans are missed. Here we show that a microhabitat composed of liquid S-medium and live bacteria in microtiter wells prolongs C. elegans longevity and is optimally suited to monitor chronic eNP-effects over the entire life span (about 34 days) of the nematode...
October 11, 2017: Environmental Pollution
https://www.readbyqxmd.com/read/29030452/longitudinal-csf-biomarkers-in-patients-with-early-parkinson-disease-and-healthy-controls
#11
Brit Mollenhauer, Chelsea J Caspell-Garcia, Christopher S Coffey, Peggy Taylor, Leslie M Shaw, John Q Trojanowski, Andy Singleton, Mark Frasier, Kenneth Marek, Douglas Galasko
OBJECTIVE: To analyze longitudinal levels of CSF biomarkers in drug-naive patients with Parkinson disease (PD) and healthy controls (HC), examine the extent to which these biomarker changes relate to clinical measures of PD, and identify what may influence them. METHODS: CSF α-synuclein (α-syn), total and phosphorylated tau (t- and p-tau), and β-amyloid 1-42 (Aβ42) were measured at baseline and 6 and 12 months in 173 patients with PD and 112 matched HC in the international multicenter Parkinson's Progression Marker Initiative...
November 7, 2017: Neurology
https://www.readbyqxmd.com/read/29027761/oxidative-stress-and-mitochondrial-dynamics-malfunction-are-linked-in-pelizaeus-merzbacher-disease
#12
Montserrat Ruiz, Mélina Bégou, Nathalie Launay, Pablo Ranea-Robles, Patrizia Bianchi, Jone López-Erauskin, Laia Morató, Cristina Guilera, Bérengère Petit, Catherine Vaurs-Barriere, Céline Guéret-Gonthier, Marie-Noëlle Bonnet-Dupeyron, Stéphane Fourcade, Johan Auwerx, Odile Boespflug-Tanguy, Aurora Pujol
Pelizaeus-Merzbacher disease (PMD) is a fatal hypomyelinating disorder characterized by early impairment of motor development, nystagmus, choreoathetotic movements, ataxia and progressive spasticity. PMD is caused by variations in the proteolipid protein gene PLP1, which encodes the two major myelin proteins of the central nervous system, PLP and its spliced isoform DM20, in oligodendrocytes. Large duplications including the entire PLP1 gene are the most frequent causative mutation leading to the classical form of PMD...
October 13, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28993467/lamtor-ragulator-is-a-negative-regulator-of-arl8b-and-borc-dependent-late-endosomal-positioning
#13
Przemyslaw A Filipek, Mariana E G de Araujo, Georg F Vogel, Cedric H De Smet, Daniela Eberharter, Manuele Rebsamen, Elena L Rudashevskaya, Leopold Kremser, Teodor Yordanov, Philipp Tschaikner, Barbara G Fürnrohr, Stefan Lechner, Theresia Dunzendorfer-Matt, Klaus Scheffzek, Keiryn L Bennett, Giulio Superti-Furga, Herbert H Lindner, Taras Stasyk, Lukas A Huber
Signaling from lysosomes controls cellular clearance and energy metabolism. Lysosomal malfunction has been implicated in several pathologies, including neurodegeneration, cancer, infection, immunodeficiency, and obesity. Interestingly, many functions are dependent on the organelle position. Lysosomal motility requires the integration of extracellular and intracellular signals that converge on a competition between motor proteins that ultimately control lysosomal movement on microtubules. Here, we identify a novel upstream control mechanism of Arl8b-dependent lysosomal movement toward the periphery of the cell...
October 9, 2017: Journal of Cell Biology
https://www.readbyqxmd.com/read/28975044/recent-updates-on-acquired-hepatocerebral-degeneration
#14
REVIEW
Hae-Won Shin, Hee Kyung Park
BACKGROUND: Acquired hepatocerebral degeneration (AHD) refers to a chronic neurological syndrome in patients with advanced hepatobiliary diseases. This comprehensive review focuses on the pathomechanism and neuroimaging findings in AHD. METHODS: A PubMed search was performed using the terms "acquired hepatocerebral degeneration," "chronic hepatocerebral degeneration," "Non-Wilsonian hepatocerebral degeneration," "cirrhosis-related parkinsonism," and "manganese and liver disease...
2017: Tremor and Other Hyperkinetic Movements
https://www.readbyqxmd.com/read/28974375/murine-sialidase-neu3-facilitates-gm2-degradation-and-bypass-in-mouse-model-of-tay-sachs-disease
#15
Volkan Seyrantepe, Secil Akyildiz Demir, Zehra Kevser Timur, Johanna Von Gerichten, Christian Marsching, Esra Erdemli, Emin Oztas, Kohta Takahashi, Kazunori Yamaguchi, Nurselin Ates, Buket Dönmez Demir, Turgay Dalkara, Katrin Erich, Carsten Hopf, Roger Sandhoff, Taeko Miyagi
Tay-Sachs disease is a severe lysosomal storage disorder caused by mutations in Hexa, the gene that encodes for the α subunit of lysosomal β-hexosaminidase A (HEXA), which converts GM2 to GM3 ganglioside. Unexpectedly, Hexa(-/-) mice have a normal lifespan and show no obvious neurological impairment until at least one year of age. These mice catabolize stored GM2 ganglioside using sialidase(s) to remove sialic acid and form the glycolipid GA2, which is further processed by β-hexosaminidase B. Therefore, the presence of the sialidase (s) allows the consequences of the Hexa defect to be bypassed...
September 30, 2017: Experimental Neurology
https://www.readbyqxmd.com/read/28973502/from-the-cover-proteome-profile-of-different-rat-brain-regions-after-sarin-intoxication
#16
Kalyani Chaubey, Syed Imteyaz Alam, Durga Prasad Nagar, Chandra Kant Waghmare, Satish C Pant, Lokendra Singh, Nalini Srivastava, Bijoy K Bhattacharya
Sarin is an organophosphorus (OP) chemical warfare agent which irreversibly inhibits acetylcholinesterase. Acute toxicity after sarin exposure is because of hyper activation of the nicotinic and muscarinic receptor. Survivors of sarin exposure often develop long-term neuropathology referred as OP ester-induced chronic neurotoxicity. However, the exact mechanism of chronic neurotoxicity is yet unknown. We studied proteomic changes in rat brain regions after 0.5 LD50 dose of sarin and investigated some milestone changes associated with long-term CNS injury...
November 1, 2017: Toxicological Sciences: An Official Journal of the Society of Toxicology
https://www.readbyqxmd.com/read/28952015/cross-talk-between-glia-neurons-and-mast-cells-in-neuroinflammation-associated-with-parkinson-s-disease
#17
Duraisamy Kempuraj, Govindhasamy Pushpavathi Selvakumar, Smita Zaheer, Ramasamy Thangavel, Mohammad Ejaz Ahmed, Sudhanshu Raikwar, Raghav Govindarajan, Shankar Iyer, Asgar Zaheer
Parkinson's disease (PD) is a progressive movement disorder characterized by neuroinflammation and dopaminergic neurodegeneration in the brain. 1-methyl-4-phenylpyridinium (MPP(+)), a metabolite of the parkinsonian neurotoxin 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) induces the release of inflammatory mediators from glial cells and neurons. Glia maturation factor (GMF), a brain proinflammatory protein, MPP(+), and mast cell-derived inflammatory mediators induce neurodegeneration which eventually leads to PD...
September 26, 2017: Journal of Neuroimmune Pharmacology: the Official Journal of the Society on NeuroImmune Pharmacology
https://www.readbyqxmd.com/read/28949038/parkinsonism-without-dopamine-neuron-degeneration-in-aged-l-dopa-responsive-dystonia-knockin-mice
#18
Samuel J Rose, Porter Harrast, Christine Donsante, Xueliang Fan, Valerie Joers, Malú G Tansey, H A Jinnah, Ellen J Hess
BACKGROUND: Recent neuroimaging studies implicate nigrostriatal degeneration as a critical factor in producing late-onset parkinsonism in patients with l-dopa-responsive dystonia-causing mutations. However, postmortem anatomical studies do not reveal neurodegeneration in l-dopa-responsive dystonia patients. These contrasting findings make it unclear how parkinsonism develops in l-dopa-responsive dystonia mutation carriers. METHODS: We prospectively assessed motor dysfunction, responses to dopaminergic challenge, and dopamine neuron degeneration with aging in a validated knockin mouse model bearing a l-dopa-responsive dystonia-causing mutation found in humans...
September 26, 2017: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/28940157/climbing-fiber-development-is-impaired-in-postnatal-car8-wdl-mice
#19
Lauren N Miterko, Roy V Sillitoe
The cerebellum is critical for an array of motor functions. During postnatal development, the Purkinje cells (PCs) guide afferent topography to establish the final circuit. Perturbing PC morphogenesis or activity during development can result in climbing fiber (CF) multi-innervation or mis-patterning. Structural defects during circuit formation typically have long-term effects on behavior as they contribute to the phenotype of movement disorders such as cerebellar ataxia. The Car8 (wdl) mouse is one model in which early circuit destruction influences movement...
September 22, 2017: Cerebellum
https://www.readbyqxmd.com/read/28931001/an-automated-rapid-iterative-negative-geotaxis-assay-for-analyzing-adult-climbing-behavior-in-a-drosophila-model-of-neurodegeneration
#20
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
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