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https://www.readbyqxmd.com/read/29305061/a-refined-concept-alpha-synuclein-dysregulation-disease
#1
REVIEW
Hideki Mochizuki, Chi-Jing Choong, Eliezer Masliah
Alpha synuclein (αSyn) still remains a mysterious protein even two decades after SNCA encoding it was identified as the first causative gene of familial Parkinson's disease (PD). Accumulation of αSyn causes α-synucleinopathies including PD, dementia with Lewy bodies (DLB) and multiple system atrophy (MSA). Recent advances in therapeutic approaches offer new antibody-, vaccine-, antisense-oligonucleotide- and small molecule-based options to reduce αSyn protein levels and aggregates in patient's brain. Gathering research information of other neurological disease particularly Alzheimer's disease, recent disappointment of an experimental amyloid plaques busting antibody in clinical trials underscores the difficulty of treating people who show even mild dementia as damage in their brain may already be too extensive...
January 2, 2018: Neurochemistry International
https://www.readbyqxmd.com/read/29298733/progressive-striatonigral-degeneration%C3%A2-in-a-transgenic-mouse-model-of-multiple-system-atrophy-translational-implications-for-interventional-therapies
#2
Violetta Refolo, Francesco Bez, Alexia Polissidis, Daniela Kuzdas-Wood, Edith Sturm, Martina Kamaratou, Werner Poewe, Leonidas Stefanis, M Angela Cenci, Marina Romero-Ramos, Gregor K Wenning, Nadia Stefanova
Multiple system atrophy (MSA) is a rapidly progressive neurodegenerative disorder characterized by widespread oligodendroglial cytoplasmic inclusions of filamentous α-synuclein, and neuronal loss in autonomic centres, basal ganglia and cerebellar circuits. It has been suggested that primary oligodendroglial α-synucleinopathy may represent a trigger in the pathogenesis of MSA, but the mechanisms underlying selective vulnerability and disease progression are unclear. The post-mortem analysis of MSA brains provides a static final picture of the disease neuropathology, but gives no clear indication on the sequence of pathogenic events in MSA...
January 3, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29297596/neuropathology-of-autonomic-dysfunction-in-synucleinopathies
#3
REVIEW
Elizabeth A Coon, Jeremy K Cutsforth-Gregory, Eduardo E Benarroch
The synucleinopathies-Parkinson's disease, dementia with Lewy bodies, multiple system atrophy, and pure autonomic failure-result from distinct patterns of abnormal α-synuclein aggregation throughout the nervous system. Autonomic dysfunction in these disorders results from variable involvement of the central and peripheral autonomic networks. The major pathologic hallmark of Parkinson's disease and dementia with Lewy bodies is Lewy bodies and Lewy neurites; of multiple system atrophy, oligodendroglial cytoplasmic inclusions; and of pure autonomic failure, peripheral neuronal cytoplasmic inclusions...
January 3, 2018: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/29281030/motor-learning-and-metaplasticity-in-striatal-neurons-relevance-for-parkinson-s-disease
#4
Nadia Giordano, Attilio Iemolo, Maria Mancini, Fabrizio Cacace, Maria De Risi, Emanuele Claudio Latagliata, Veronica Ghiglieri, Gian Carlo Bellenchi, Stefano Puglisi-Allegra, Paolo Calabresi, Barbara Picconi, Elvira De Leonibus
Nigro-striatal dopamine transmission is central to a wide range of neuronal functions, including skill learning, which is disrupted in several pathologies such as Parkinson's disease. The synaptic plasticity mechanisms, by which initial motor learning is stored for long time periods in striatal neurons, to then be gradually optimized upon subsequent training, remain unexplored. Addressing this issue is crucial to identify the synaptic and molecular mechanisms involved in striatal-dependent learning impairment in Parkinson's disease...
December 21, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29259548/differential-effects-of-parkinson-s-disease-on-interneuron-subtypes-within-the-human-anterior-olfactory-nucleus
#5
Isabel Ubeda-Bañon, Alicia Flores-Cuadrado, Daniel Saiz-Sanchez, Alino Martinez-Marcos
Synucleinopathies (including α-synucleinopathies), which include Parkinson's disease (PD), manifest themsevles early on (stage 1) in the olfactory system; preferentially in the anterior olfactory nucleus (AON). In particular, the non-motor, early manifestations of PD include hyposmia, which is the partial loss of the sense of smell. The neural basis of hyposmia in PD, however, is poorly understood; but the AON appears to be a key structure in the disease's progression. We analyzed whether α-synuclein was involved in the differential interneuron vulnerability associated with PD in the retrobulbar, cortical anterior and cortical posterior divisions of the AON...
2017: Frontiers in Neuroanatomy
https://www.readbyqxmd.com/read/29249285/%C3%AE-synuclein-induces-mitochondrial-dysfunction-through-spectrin-and-the-actin-cytoskeleton
#6
Dalila G Ordonez, Michael K Lee, Mel B Feany
Genetics and neuropathology strongly link α-synuclein aggregation and neurotoxicity to the pathogenesis of Parkinson's disease and related α-synucleinopathies. Here we describe a new Drosophila model of α-synucleinopathy based on widespread expression of wild-type human α-synuclein, which shows robust neurodegeneration, early-onset locomotor deficits, and abundant α-synuclein aggregation. We use results of forward genetic screening and genetic analysis in our new model to demonstrate that α-synuclein expression promotes reorganization of the actin filament network and consequent mitochondrial dysfunction through altered Drp1 localization...
December 13, 2017: Neuron
https://www.readbyqxmd.com/read/29246926/combined-active-humoral-and-cellular-immunization-approaches-for-the-treatment-of-synucleinopathies
#7
Edward Rockenstein, Gary Ostroff, Fusun Dikengil, Florentina Rus, Michael Mante, Jazmin Florio, Anthony Adame, Ivy Trinh, Changyoun Kim, Cassia Overk, Eliezer Masliah, Robert A Rissman
Dementia with Lewy bodies (DLB), Parkinson's disease (PD) and Multiple System Atrophy (MSA) are age-related neurodegenerative disorders characterized by progressive accumulation of α-synuclein (α-syn) and jointly termed synucleinopathies. Currently, no disease-modifying treatments are available for these disorders. Previous preclinical studies showed that active and passive immunizations targeting α-syn partially ameliorates behavioral deficits and α-syn accumulation; however, it is unknown if combining humoral and cellular immunization might act synergistically to reduce inflammation and improve microglial-mediated α-syn clearance...
December 15, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/29246765/fkbp12-immunopositive-inclusions-in-patients-with-%C3%AE-synucleinopathies
#8
Yasuyuki Honjo, Takashi Ayaki, Tomohisa Horibe, Hidefumi Ito, Ryosuke Takahashi, Koji Kawakami
α-Synuclein (α-SYN), a presynaptic protein with the tendency to aggregate, is linked to α-synucleinopathies such as Parkinson's disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA). α-SYN is the main component of round intracytoplasmic inclusions called Lewy bodies (LBs), which are the hallmark of PD and DLB. In addition, accumulation of amyloid-β and neurofibrillary tangles as in the pathology of Alzheimer's disease has been found in the DLB brain. Glial cytoplasmic inclusions are an MSA-specific type of inclusion found in oligodendrocytes and mainly comprise α-SYN...
December 12, 2017: Brain Research
https://www.readbyqxmd.com/read/29246724/toxic-properties-of-microsome-associated-alpha-synuclein-species-in-mouse-primary-neurons
#9
Emanuela Colla, Giulia Panattoni, Alessio Ricci, Caterina Rizzi, Lucia Rota, Nicola Carucci, Verdiana Valvano, Francesco Gobbo, Simona Capsoni, Michael K Lee, Antonino Cattaneo
α-synuclein (αS) is a small protein that self-aggregates into α-helical oligomer species and subsequently into larger insoluble amyloid fibrils that accumulate in intraneuronal inclusions during the development of Parkinson's disease. Toxicity of αS oligomers and fibrils has been long debated and more recent data are suggesting that both species can induce neurodegeneration. However while most of these data are based on differences in structure between oligomer and aggregates, often preassembled in vitro, the in vivo situation might be more complex and subcellular locations where αS species accumulate, rather than their conformation, might contribute to enhanced toxicity...
December 12, 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/29236831/pontine-calcification-in-late-stage-cerebellar-multiple-system-atrophy-a-marker-of-synucleinopathy-neurodegeneration
#10
José Luiz Pedroso, Orlando G Barsottini, Marcos Knobel, Benjamin Wolf Handfas, Antonio J Rocha, Elias Knobel
No abstract text is available yet for this article.
November 2017: Arquivos de Neuro-psiquiatria
https://www.readbyqxmd.com/read/29232183/loss-of-substantia-nigra-hyperintensity-at-3-0-t-mr-imaging-in-idiopathic-rem-sleep-behavior-disorder-comparison-with-123i-fp-cit-spect
#11
Yun Jung Bae, Jong-Min Kim, Kyeong Joon Kim, Eunhee Kim, Hyun Soo Park, Seo Young Kang, In-Young Yoon, Jee-Young Lee, Beomseok Jeon, Sang Eun Kim
Purpose To examine whether the loss of nigral hyperintensity (NH) on 3.0-T susceptibility-weighted (SW) magnetic resonance (MR) images can help identify high synucleinopathy risk in patients with idiopathic rapid eye movement sleep behavior disorder (iRBD). Materials and Methods Between March 2014 and April 2015, 18 consecutively recruited patients with iRBD were evaluated with 3.0-T SW imaging and iodine 123-2β-carbomethoxy-3β-(4-iodophenyl)-N-(3-fluoropropyl)-nortropane (123I-FP-CIT) single photon emission computed tomography and compared with 18 healthy subjects and 18 patients with Parkinson disease (PD)...
December 12, 2017: Radiology
https://www.readbyqxmd.com/read/29229294/impact-of-dha-intake-in-a-mouse-model-of-synucleinopathy
#12
Katherine Coulombe, Olivier Kerdiles, Cyntia Tremblay, Vincent Emond, Manon Lebel, Anne-Sophie Boulianne, Mélanie Plourde, Francesca Cicchetti, Frédéric Calon
Polyunsaturated fatty acids omega-3 (n-3 PUFA), such as docosahexaenoic acid (DHA), have been shown to prevent, and partially reverse, neurotoxin-induced nigrostriatal denervation in animal models of Parkinson's disease (PD). However, the accumulation of α-synuclein (αSyn) in cerebral tissues is equally important to the pathophysiology. To determine whether DHA intake improves various aspects related to synucleinopathy, ninety male mice overexpressing human αSyn under the Thy-1 promoter (Thy1-αSyn) were fed one of three diets (specially formulated control, low n-3 PUFA or high DHA) and compared to non-transgenic C57/BL6 littermate mice exposed to a control diet...
December 8, 2017: Experimental Neurology
https://www.readbyqxmd.com/read/29225795/multiple-system-atrophy-genetic-risks-and-alpha-synuclein-mutations
#13
REVIEW
Heather T Whittaker, Yichen Qui, Conceição Bettencourt, Henry Houlden
Multiple system atrophy (MSA) is one of the few neurodegenerative disorders where we have a significant understanding of the clinical and pathological manifestations but where the aetiology remains almost completely unknown. Research to overcome this hurdle is gaining momentum through international research collaboration and a series of genetic and molecular discoveries in the last few years, which have advanced our knowledge of this rare synucleinopathy. In MSA, the discovery of α-synuclein pathology and glial cytoplasmic inclusions remain the most significant findings...
2017: F1000Research
https://www.readbyqxmd.com/read/29222591/dementia-with-lewy-bodies-and-parkinson-s-disease-dementia-current-concepts-and-controversies
#14
REVIEW
Kurt A Jellinger
Dementia with Lewy bodies (DLB) and Parkinson's disease-dementia (PDD), although sharing many clinical, neurochemical and morphological features, according to DSM-5, are two entities of major neurocognitive disorders with Lewy bodies of unknown etiology. Despite considerable clinical overlap, their diagnosis is based on an arbitrary distinction between the time of onset of motor and cognitive symptoms: dementia often preceding parkinsonism in DLB and onset of cognitive impairment after onset of motor symptoms in PDD...
December 8, 2017: Journal of Neural Transmission
https://www.readbyqxmd.com/read/29217261/synucleinopathies-in-neurodegenerative-diseases-accomplices-an-inside-job-and-selective-vulnerability
#15
S Ausim Azizi, Saara-Anne Azizi
Pathogenesis of degenerative diseases is complex and multifaceted. The disease phenotypes depend on the location of injury/damage in the brain networks and pathologically are characterized by loss of brain cells. The reason for this loss appears to be an accumulation of misfolded and dysfunctional proteins that trigger apoptotic cell death. The role of alpha-synuclein mutations, its interaction with other proteins and the cellular environment is discussed in the context of selective neuron loss.
December 4, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/29216391/prevalence-and-determinants-of-rem-sleep-behavior-disorder-in-the-general-population
#16
José Haba-Rubio, Birgit Frauscher, Pedro Marques-Vidal, Jérôme Toriel, Nadia Tobback, Daniela Andries, Martin Preisig, Peter Vollenweider, Ronald Postuma, Raphaël Heinzer
Study Objectives: REM sleep behavior disorder (RBD) is a parasomnia associated with neurodegenerative synucleinopathies. Its prevalence is largely unknown. This study determined the prevalence and characteristics of RBD in the general population using gold-standard polysomnography. Methods: Full polysomnographic data from 1997 subjects (age=59±11.1 years, 53.6% women) participating in a population-based study (HypnoLaus, Lausanne, Switzerland) were collected. Sleep-related complaints and habits were investigated using various sleep measures including the Munich Parasomnia Screening (MUPS) questionnaire, which includes two questions evaluating complex motor behaviors suggestive of RBD...
December 5, 2017: Sleep
https://www.readbyqxmd.com/read/29214093/should-skin-biopsies-be-performed-in-patients-suspected-of-having-parkinson-s-disease
#17
REVIEW
Timo Siepmann, Ana Isabel Penzlin, Ben Min-Woo Illigens, Heinz Reichmann
In patients with Parkinson's disease (PD), the molecularly misfolded form of α-synuclein was recently identified in cutaneous autonomic nerve fibers which displayed increased accumulation even in early disease stages. However, the underlying mechanisms of synucleinopathic nerve damage and its implication for brain pathology in later life remain to be elucidated. To date, specific diagnostic tools to evaluate small fiber pathology and to discriminate neurodegenerative proteinopathies are rare. Recently, research has indicated that deposition of α-synuclein in cutaneous nerve fibers quantified via immunohistochemistry in superficial skin biopsies might be a valid marker of PD which could facilitate early diagnosis and monitoring of disease progression...
2017: Parkinson's Disease
https://www.readbyqxmd.com/read/29209768/spread-of-aggregates-after-olfactory-bulb-injection-of-%C3%AE-synuclein-fibrils-is-associated-with-early-neuronal-loss-and-is-reduced-long-term
#18
Nolwen L Rey, Sonia George, Jennifer A Steiner, Zachary Madaj, Kelvin C Luk, John Q Trojanowski, Virginia M-Y Lee, Patrik Brundin
Parkinson's disease is characterized by degeneration of substantia nigra dopamine neurons and by intraneuronal aggregates, primarily composed of misfolded α-synuclein. The α-synuclein aggregates in Parkinson's patients are suggested to first appear in the olfactory bulb and enteric nerves and then propagate, following a stereotypic pattern, via neural pathways to numerous regions across the brain. We recently demonstrated that after injection of either mouse or human α-synuclein fibrils into the olfactory bulb of wild-type mice, α-synuclein fibrils recruited endogenous α-synuclein into pathological aggregates that spread transneuronally to over 40 other brain regions and subregions, over 12 months...
December 5, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/29203207/suppression-of-autophagy-in-the-brain-of-transgenic-mice-with-overexpression-of-%C3%B0-53%C3%B0-mutant-%C3%AE-synuclein-as-an-early-event-at-synucleinopathy-progression
#19
Alexander B Pupyshev, Tatiana A Korolenko, Anna A Akopyan, Tamara G Amstislavskaya, Maria A Tikhonova
Transgenic overexpression of α-synuclein is a common model of Parkinson's disease (PD). Accumulation of А53Т-mutant α-synuclein induces three autophagy cell response: the inhibition of autophagy caused by the accumulation of α-synuclein, compensatory activation of macroautophagy in response to inhibition of the CMA, and toxic effects of mutant α-synuclein accompanied by the activation of autophagy. The overall effect of long-term overexpression of mutant α-synuclein in vivo remained unclear. Here we evaluated the activity of autophagy in the frontal cortex, striatum and s...
December 1, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/29198173/autophagy-inhibition-promotes-snca-alpha-synuclein-release-and-transfer-via-extracellular-vesicles-with-a-hybrid-autophagosome-exosome-like-phenotype
#20
Georgia Minakaki, Stefanie Menges, Agnes Kittel, Evangelia Emmanouilidou, Iris Schaeffner, Katalin Barkovits, Anna Bergmann, Edward Rockenstein, Anthony Adame, Franz Marxreiter, Brit Mollenhauer, Douglas Galasko, Edit Irén Buzás, Ursula Schlötzer-Schrehardt, Katrin Marcus, Wei Xiang, Dieter Chichung Lie, Kostas Vekrellis, Eliezer Masliah, Jürgen Winkler, Jochen Klucken
The autophagy-lysosome pathway (ALP) regulates intracellular homeostasis of the cytosolic protein SNCA/alpha-synuclein and is impaired in synucleinopathies, including Parkinson disease and dementia with Lewy bodies (DLB). Emerging evidence suggests that ALP influences SNCA release, but the underlying cellular mechanisms are not well understood. Several studies identified SNCA in exosome/extracellular vesicle (EV) fractions. EVs are generated in the multivesicular body compartment and either released upon its fusion with the plasma membrane, or cleared via the ALP...
December 4, 2017: Autophagy
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