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multiple system atrophy

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https://www.readbyqxmd.com/read/28931065/chlamydomonas-fap265-is-a-tubulin-polymerization-promoting-protein-essential-for-flagellar-reassembly-and-hatching-of-daughter-cells-from-the-sporangium
#1
Damayanti Tammana, Trinadh Venkata Satish Tammana
Tubulin polymerization promoting proteins (TPPPs) belong to a family of neomorphic moon lighting proteins, involved in various physiological and pathological conditions. In physiological conditions, TPPPs play an important role in microtubule dynamics regulating mitotic spindle assembly and in turn cell proliferation. In pathological situations, TPPPs interact with α-synuclein and β-amyloid and promote their aggregation leading to Parkinson's disease and multiple system atrophy. Orthologs of TPPP family proteins were identified in ciliary proteomes from various organisms including Chlamydomonas but their role in ciliogenesis was not known...
2017: PloS One
https://www.readbyqxmd.com/read/28922189/acr-acnm-practice-parameter-for-the-performance-of-dopamine-transporter-dat-single-photon-emission-computed-tomography-spect-imaging-for-movement-disorders
#2
Rathan M Subramaniam, Kirk A Frey, Christopher H Hunt, Gustavo A Mercier, Lilja B Solnes, Patrick M Colletti, Yang Lu, Bital Savir-Baruch, Hadyn T Williams
This American College of Radiology and American College of Nuclear Medicine joint clinical practice parameter is for performance of dopamine transporter single photon emission computed tomography (SPECT) imaging, for patients with movement disorders. Parkinsonian syndrome (PS) consists of a group of neurodegenerative diseases including Parkinson disease (PD), progressive supranuclear palsy (PSP), multiple system atrophy (MSA), corticobasal degeneration (CBD), and dementia with Lewy bodies (DLB). Accurate diagnosis of PS is critical for clinical management...
September 15, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28912150/-18-f-fdg-pet-in-parkinsonism-differential-diagnosis-and-cognitive-impairment-in-parkinson-s-disease
#3
Philipp T Meyer, Lars Frings, Gerta Rücker, Sabine Hellwig
Accurate differential diagnosis of parkinsonism is of a paramount therapeutic and prognostic importance. In addition, with the development of invasive therapies and novel disease-specific therapies, strategies for patient enrichment in trial populations are of growing importance. Imaging disease-specific patterns of regional glucose metabolism with positron emission tomography and [(18)F]fluoro-2-deoxy-2-D-glucose ((18)F-FDG) allows for a highly accurate distinction between Parkinson's disease (PD) and atypical parkinsonian syndromes (APS) including multiple system atrophy (MSA), progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD)...
September 14, 2017: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
https://www.readbyqxmd.com/read/28904388/protective-efficacy-of-phosphodiesterase-1-inhibition-against-alpha-synuclein-toxicity-revealed-by-compound-screening-in-luhmes-cells
#4
Matthias Höllerhage, Claudia Moebius, Johannes Melms, Wei-Hua Chiu, Joachim N Goebel, Tasnim Chakroun, Thomas Koeglsperger, Wolfgang H Oertel, Thomas W Rösler, Marc Bickle, Günter U Höglinger
α-synuclein-induced neurotoxicity is a core pathogenic event in neurodegenerative synucleinopathies such as Parkinson's disease, dementia with Lewy bodies, or multiple system atrophy. There is currently no disease-modifying therapy available for these diseases. We screened 1,600 FDA-approved drugs for their efficacy to protect LUHMES cells from degeneration induced by wild-type α-synuclein and identified dipyridamole, a non-selective phosphodiesterase inhibitor, as top hit. Systematic analysis of other phosphodiesterase inhibitors identified a specific phosphodiesterase 1 inhibitor as most potent to rescue from α-synuclein toxicity...
September 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28899515/the-water-extract-of-liuwei-dihuang-possesses-multi-protective-properties-on-neurons-and-muscle-tissue-against-deficiency-of-survival-motor-neuron-protein
#5
Yu-Ting Tseng, Yuh-Jyh Jong, Wei-Fang Liang, Fang-Rong Chang, Yi-Ching Lo
BACKGROUND: Deficiency of survival motor neuron (SMN) protein, which is encoded by the SMN1 and SMN2 genes, induces widespread splicing defects mainly in spinal motor neurons, and leads to spinal muscular atrophy (SMA). Currently, there is no effective treatment for SMA. Liuwei dihuang (LWDH), a traditional Chinese herbal formula, possesses multiple therapeutic benefits against various diseases via modulation of the nervous, immune and endocrine systems. Previously, we demonstrated water extract of LWDH (LWDH-WE) protects dopaminergic neurons and improves motor activity in models of Parkinson's disease...
October 15, 2017: Phytomedicine: International Journal of Phytotherapy and Phytopharmacology
https://www.readbyqxmd.com/read/28886909/quantitative-susceptibility-mapping-differentiates-between-parkinsonian-disorders
#6
Henrik Sjöström, Tobias Granberg, Eric Westman, Per Svenningsson
INTRODUCTION: It is often challenging to clinically distinguish between Parkinson's disease (PD), multiple system atrophy (MSA) and progressive supranuclear palsy (PSP). Quantitative susceptibility mapping (QSM) is an accurate indirect method for estimating brain iron levels in vivo. This method has yet to be applied in atypical parkinsonism. We aimed to investigate differences in brain iron accumulation parkinsonian disorders and healthy controls using QSM. METHODS: 15 patients with PSP, 11 patients with MSA, 62 patients with PD and 14 healthy controls were included in the study and their phase and magnitude data from susceptibility-weighted magnetic resonance imaging were retrospectively analyzed with an in-house pipeline to create susceptibility maps...
September 1, 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/28882381/trunk-muscle-activation-pattern-in-parkinsonian-camptocormia-as-revealed-with-surface-electromyography
#7
Nils G Margraf, Marten Rogalski, Günther Deuschl, Johann P Kuhtz-Buschbeck
INTRODUCTION: Camptocormia is frequently seen in Parkinson's disease (PD) and multiple system atrophy. It is characterized by a pathological forward bending of the trunk during standing, often combined with a lateral trunk deviation. The etiology of camptocormia in PD is still unknown. Muscle MRI studies show abnormalities mainly of the erector spinae confirmed by muscle biopsies. Quantitative electromyographic examination of trunk muscle activity is missing. METHODS: Ventral (rectus and obliquus externus abdominis) and dorsal (iliocostalis lumborum, longissimus, multifidus) trunk muscles and the rectus femoris were recorded bilaterally with surface electromyography in standing PD patients with camptocormia (n = 10) and matched healthy controls (n = 10) who mimicked the patients' posture...
August 31, 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/28879902/review-of-tremor-in-parkinson-s-disease-and-atypical-parkinsonian-disorders
#8
Pooja Mailankody, M Netravathi, Pramod Kumar Pal
Rest tremor (RT), a cardinal feature of Parkinson's disease (PD) is often accompanied by other types of tremor such as action tremor, which includes postural tremor, kinetic tremor, re-emergent tremor (ReT), and orthostatic tremor (OT). Literature on other tremors of PD, especially ReT and OT, is scarce. Tremor can be present in any of the atypical parkinsonian disorders such as progressive supranuclear palsy, multiple system atrophy, corticobasal degeneration, and dementia with Lewy bodies. RT can even be the presenting symptom of these disorders...
September 2017: Neurology India
https://www.readbyqxmd.com/read/28877271/spinal-cord-homogenates-from-sod1-familial-amyotrophic-lateral-sclerosis-induce-sod1-aggregation-in-living-cells
#9
Edward Pokrishevsky, Ran Ha Hong, Ian R Mackenzie, Neil R Cashman
Mutant Cu/Zn superoxide dismutase (SOD1) can confer its misfolding on wild-type SOD1 in living cells; the propagation of misfolding can also be transmitted between cells in vitro. Recent studies identified fluorescently-tagged SOD1G85R as a promiscuous substrate that is highly prone to aggregate by a variety of templates, in vitro and in vivo. Here, we utilized several SOD1-GFP reporter proteins with G37R, G85R, or G93A mutations in SOD1. We observed that human spinal cord homogenates prepared from SOD1 familial ALS (FALS) can induce significantly more intracellular reporter protein aggregation than spinal cord homogenates from sporadic ALS, Alzheimer's disease, multiple system atrophy or healthy control individuals...
2017: PloS One
https://www.readbyqxmd.com/read/28870566/pre-and-postganglionic-vasomotor-dysfunction-causes-distal-limb-coldness-in-multiple-system-atrophy
#10
Kazumasa Shindo, Mai Tsuchiya, Yuta Ichinose, Kishin Koh, Takanori Hata, Nobuo Yamashiro, Fumikazu Kobayashi, Takamura Nagasaka, Yoshihisa Takiyama
BACKGROUND: The detailed pathophysiology of limb coldness in multiple system atrophy (MSA) is unknown. METHODS: We evaluated cutaneous vasomotor neural function in 18 MSA patients with or without limb coldness, and in 20 healthy volunteers as controls. We measured resting skin sympathetic nerve activity (SSNA) and spontaneous changes of the sympathetic skin response (SSR) and skin blood flow (skin vasomotor reflex: SVR), as well as SVR and reflex changes of SSNA after electrical stimulation...
September 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28867372/key-themes-and-future-prospects-in-translational-multiple-system-atrophy-research
#11
REVIEW
S Eschlboeck, F Krismer, G K Wenning
Multiple system atrophy (MSA) is a rapidly progressive neurodegenerative disorder with a highly variable clinical presentation. Unfortunately, there exists no effective therapy that can improve the course of the disease and symptomatic treatment options remain limited. Although significant progress in research has improved our understanding of MSA, knowledge gaps still remain. Thus, a global network focusing on different research areas is required to face this fatal disease.
August 12, 2017: Autonomic Neuroscience: Basic & Clinical
https://www.readbyqxmd.com/read/28860330/recent-advances-in-neuropathology-biomarkers-and-therapeutic-approach-of-multiple-system-atrophy
#12
REVIEW
Shunsuke Koga, Dennis W Dickson
Multiple system atrophy (MSA) is a progressive neurodegenerative disorder characterised by a variable combination of autonomic failure, levodopa-unresponsive parkinsonism, cerebellar ataxia and pyramidal symptoms. The pathological hallmark is the oligodendrocytic glial cytoplasmic inclusion (GCI) consisting of α-synuclein; therefore, MSA is included in the category of α-synucleinopathies. MSA has been divided into two clinicopathological subtypes: MSA with predominant parkinsonism and MSA with predominant cerebellar ataxia, which generally correlate with striatonigral degeneration and olivopontocerebellar atrophy, respectively...
August 31, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28854775/gastrocolocutaneous-fistula-an-unusual-case-of-gastrostomy-tube-malfunction-with-diarrhea
#13
Junghwan Lee, Jinyoung Kim, Ha Il Kim, Chung Ryul Oh, Sungim Choi, Soomin Noh, Hee Kyong Na, Hwoon-Yong Jung
A gastrocolocutaneous fistula is a rare complication of percutaneous endoscopic gastrostomy (PEG). We report a case of a gastrocolocutaneous fistula presenting with intractable diarrhea and gastrostomy tube malfunction. A 62-year-old woman with a history of multiple system atrophy was referred to us because of PEG tube malfunction. Twenty days prior to presentation, the patient started developing sudden diarrhea within minutes after starting PEG feeding. Fluoroscopy revealed that the balloon of the PEG tube was located in the lumen of the transverse colon with the contrast material filling the colon...
August 31, 2017: Clinical Endoscopy
https://www.readbyqxmd.com/read/28845583/editorial-comment-to-case-of-possible-multiple-system-atrophy-with-a-characteristic-imaging-finding-of-open-bladder-neck-during-storage-phase-as-an-initial-sign
#14
Ryuji Sakakibara
No abstract text is available yet for this article.
August 28, 2017: International Journal of Urology: Official Journal of the Japanese Urological Association
https://www.readbyqxmd.com/read/28844067/cerebellar-lobule-atrophy-and-disability-in-progressive-ms
#15
Sirio Cocozza, Maria Petracca, Enricomaria Mormina, Korhan Buyukturkoglu, Kornelius Podranski, Monika M Heinig, Giuseppe Pontillo, Camilla Russo, Enrico Tedeschi, Cinzia Valeria Russo, Teresa Costabile, Roberta Lanzillo, Asaff Harel, Sylvia Klineova, Aaron Miller, Arturo Brunetti, Vincenzo Brescia Morra, Fred Lublin, Matilde Inglese
OBJECTIVE: To investigate global and lobular cerebellar volumetries in patients with progressive multiple sclerosis (MS), testing the contribution of cerebellar lobular atrophy to both motor and cognitive performances. METHODS: Eighty-two patients with progressive MS and 46 healthy controls (HC) were enrolled in this cross-sectional study. Clinical evaluation included motor and cognitive testing: Expanded Disability Status Scale, cerebellar Functional System score, Timed 25-Foot Walk Test, 9-Hole Peg Test (9-HPT), Symbol Digit Modalities Test (SDMT), Brief Visuospatial Memory Test-Revised (BVMT) and California Verbal Learning Test II (CVLT)...
August 26, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28833467/dopamine-transporter-imaging-deficit-predicts-early-transition-to-synucleinopathy-in-idiopathic-rem-sleep-behavior-disorder
#16
Alex Iranzo, Joan Santamaría, Francesc Valldeoriola, Monica Serradell, Manel Salamero, Carles Gaig, Aida Niñerola-Baizan, Raquel Sánchez-Valle, Albert Lladó, Roberto De Marzi, Ambra Stefani, Klaus Seppi, Javier Pavia, Birgit Högl, Werner Poewe, Eduard Tolosa, Francesc Lomeña
OBJECTIVE: To determine the usefulness of dopamine transporter (DAT) imaging to identify idiopathic REM sleep behavior disorder (IRBD) patients at risk for short-term development of clinically-defined synucleinopathy. METHODS: Eighty-seven patients with polysomnography-confirmed IRBD underwent (123) I-FP-CIT DAT-SPECT. Results were compared with 20 matched controls without RBD who underwent DAT-SPECT. In patients, FP-CIT uptake was considered abnormal when values were two standard deviations below controls' mean uptake...
August 22, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28831921/in-silico-studies-in-drug-research-against-neurodegenerative-diseases
#17
Farahnaz Rezaei Makhouri, Jahan B Ghasemi
Neurodegenerative diseases such as Alzheimer's disease (AD), progressive neurodegenerative forms of Huntington's disease, Parkinson's disease (PD), amyotrophic lateral sclerosis, spinal cerebellar ataxias, and spinal and bulbar muscular atrophy are described by slow and selective dysfunction and degeneration of neurons and axons in the central nervous system (CNS). Computer-aided or in silico design methods have matured into powerful tools for reducing the number of ligands that should be screened in experimental assays...
August 22, 2017: Current Neuropharmacology
https://www.readbyqxmd.com/read/28829981/pain-processing-in-atypical-parkinsonisms-and-parkinson-disease-a-comparative-neurophysiological-study
#18
Micol Avenali, Cristina Tassorelli, Roberto De Icco, Armando Perrotta, Mariano Serrao, Mauro Fresia, Claudio Pacchetti, Giorgio Sandrini
OBJECTIVE: Pain is a frequent non-motor feature in Parkinsonism but mechanistic data on the alteration of pain processing are insufficient to understand the possible causes and to define specifically-targeted treatments. METHODS: we investigated spinal nociception through the neurophysiological measure of the threshold (TR) of nociceptive withdrawal reflex (NWR) and its temporal summation threshold (TST) comparatively in 12 Progressive Supranuclear Palsy (PSP) subjects, 11 Multiple System Atrophy (MSA) patients, 15 Parkinson's disease (PD) subjects and 24 healthy controls (HC)...
July 17, 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28829427/recombinant-%C3%AE-%C3%AE-and-%C3%AE-synucleins-stimulate-protein-phosphatase-2a-catalytic-subunit-activity-in-cell-free-assays
#19
Sovanarak Lek, Javier Vargas-Medrano, Ernesto Villanueva, Brian Marcus, Wesley Godfrey, Ruth G Perez
α-Synuclein (aSyn), β-Synuclein (bSyn), and γ-Synuclein (gSyn) are members of a conserved family of chaperone-like proteins that are highly expressed in vertebrate neuronal tissues. Of the three synucleins, only aSyn has been strongly implicated in neurodegenerative disorders such as Parkinson's disease, Dementia with Lewy Bodies, and Multiple System Atrophy. In studying normal aSyn function, data indicate that aSyn stimulates the activity of the catalytic subunit of an abundantly expressed dephosphorylating enzyme, PP2Ac in vitro and in vivo...
August 13, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/28826526/small-molecule-pet-tracers-for-imaging-proteinopathies
#20
REVIEW
Chester A Mathis, Brian J Lopresti, Milos D Ikonomovic, William E Klunk
In this chapter, we provide a review of the challenges and advances in developing successful PET imaging agents for 3 major types of aggregated amyloid proteins: amyloid-beta (Aβ), tau, and alpha-synuclein (α-syn). These 3 amyloids are involved in the pathogenesis of a variety of neurodegenerative diseases, referred to as proteinopathies or proteopathies, that include Alzheimer disease, Lewy body dementias, multiple system atrophy, and frontotemporal dementias, among others. In the Introduction section, we briefly discuss the history of amyloid in neurodegenerative diseases and describe why progress in developing effective imaging agents has been hampered by the failure of crystallography to provide definitive ligand-protein interactions for rational radioligand design efforts...
September 2017: Seminars in Nuclear Medicine
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