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https://www.readbyqxmd.com/read/27911312/increased-transforming-growth-factor-%C3%AE-2-in-the-neocortex-of-alzheimer-s-disease-and-dementia-with-lewy-bodies-is%C3%A2-correlated-with-disease-severity-and%C3%A2-soluble-a%C3%AE-42-load
#1
Joyce R Chong, Yuek Ling Chai, Jasinda H Lee, David Howlett, Johannes Attems, Clive G Ballard, Dag Aarsland, Paul T Francis, Christopher P Chen, Mitchell K P Lai
BACKGROUND: Of the three transforming growth factor (TGF)-β isoforms known, TGFβ1 deficits have been widely reported in Alzheimer's disease (AD) and studied as a potential therapeutic target. In contrast, the status of TGFβ2, which has been shown to mediate amyloid-β (Aβ)-mediated neuronal death, are unclear both in AD and in Lewy body dementias (LBD) with differential neuritic plaque and neurofibrillary tangle burden. OBJECTIVE: To measure neocortical TGFβ2 levels and their correlations with neuropathological and clinical markers of disease severity in a well-characterized cohort of AD as well as two clinical subtypes of LBD, dementia with Lewy bodies (DLB) and Parkinson's disease dementia (PDD), known to manifest relatively high and low Aβ plaque burden, respectively...
November 28, 2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27911022/membrane-defects-and-genetic-redundancy-are-we-at-a-turning-point-for-dyt1-dystonia
#2
REVIEW
Ana Cascalho, Julie Jacquemyn, Rose E Goodchild
Heterozygosity for a 3-base pair deletion (ΔGAG) in TOR1A/torsinA is one of the most common causes of hereditary dystonia. In this review, we highlight current understanding of how this mutation causes disease from research spanning structural biochemistry, cell science, neurobiology, and several model organisms. We now know that homozygosity for ΔGAG has the same effects as Tor1a(KO) , implicating a partial loss of function mechanism in the ΔGAG/+ disease state. In addition, torsinA loss specifically affects neurons in mice, even though the gene is broadly expressed, apparently because of differential expression of homologous torsinB...
December 2, 2016: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/27878377/relationship-between-the-plasma-levels-of-neurodegenerative-proteins-and-motor-subtypes-of-parkinson-s-disease
#3
Jian Ding, Jiejin Zhang, Xixi Wang, Li Zhang, Siming Jiang, Yongsheng Yuan, Junyi Li, Lin Zhu, Kezhong Zhang
The aim of our study is to examine the plasma levels of the four kinds of neurodegenerative proteins in plasma: α-syn, T-tau, P-tau181, and Aβ-42 in Parkinson's disease (PD) and to evaluate the relationship between their plasma levels and PD motor subtypes. 84 patients with PD were enrolled in our study, and finally, 73 of them were classified into the tremor-dominant subtype (TD) and the postural instability gait difficulty subtype (PIGD). Their motor performance was evaluated by a series of clinical assessments: Freezing of Gait Questionnaire (FOGQ), Timed Up and Go (TUGs), Tinetti balance, and Tinetti gait...
November 22, 2016: Journal of Neural Transmission
https://www.readbyqxmd.com/read/27872019/metabotropic-glutamate-receptors-and-neurodegenerative-diseases
#4
REVIEW
Fabiola M Ribeiro, Luciene B Vieira, Rita G W Pires, Roenick P Olmo, Stephen S G Ferguson
Glutamate is the most important excitatory neurotransmitter of the mammalian central nervous system (CNS), playing an important role in memory, synaptic plasticity and neuronal development. However, glutamate overstimulation is also implicated in neuronal cell death. There are two major types of glutamate receptors: ionotropic and metabotropic. Thus far, eight metabotropic glutamate receptors (mGluRs) subtypes have been characterized and are divided into three subgroups based on sequence homology and cell signaling activation...
November 18, 2016: Pharmacological Research: the Official Journal of the Italian Pharmacological Society
https://www.readbyqxmd.com/read/27871435/resting-state-functional-connectivity-of-subthalamic-nucleus-in-different-parkinson-s-disease-phenotypes
#5
Zhan Wang, Huimin Chen, Huizi Ma, Lingyan Ma, Tao Wu, Tao Feng
Previous studies showed that the subthalamic nucleus (STN) plays a crucial role in Parkinson's disease (PD) pathophysiology. During rest, PD phenotypes exhibit different STN functional connectivity. STN functional connectivity was examined in 31 PD patients [12 tremor-dominant (TD) and 19 posture instability gait difficulty (PIGD)] and 22 healthy controls (HC). Compared with controls and PIGD patients, the TD patients exhibited higher functional connectivity between the bilateral STN and the left cerebellar anterior lobe...
December 15, 2016: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/27862270/familial-aggregation-of-parkinson-s-disease-may-affect-progression-of-motor-symptoms-and-dementia
#6
REVIEW
Johannes Jernqvist Gaare, Geir Olve Skeie, Charalampos Tzoulis, Jan Petter Larsen, Ole-Bjørn Tysnes
BACKGROUND: Familial aggregation has been described in PD of both early and late onset, but has not been studied in a true population-based sample. Moreover, little is known about its association with disease progression and endophenotypes. OBJECTIVES: The objectives of this work were to determine familial aggregation of idiopathic PD in a population-based cohort and study the association with clinical endophenotypes and disease progression. METHODS: We examined family history data from the Norwegian ParkWest study, a well-characterized, population-based cohort of incident PD patients and age-matched healthy controls...
November 10, 2016: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/27859565/anhidrosis-in-multiple-system-atrophy-involves-pre-and-postganglionic-sudomotor-dysfunction
#7
Elizabeth A Coon, Robert D Fealey, David M Sletten, Jay N Mandrekar, Eduardo E Benarroch, Paola Sandroni, Phillip A Low, Wolfgang Singer
BACKGROUND: The objective of this study was to characterize the degree, pattern, lesion site, and temporal evolution of sudomotor dysfunction in multiple system atrophy (MSA) and to evaluate differences by parkinsonian (MSA-parkinsonism) and cerebellar (MSA-cerebellar) subtypes. METHODS: All cases of MSA evaluated at Mayo Clinic Rochester between 2005 and 2010 with postganglionic sudomotor testing and thermoregulatory sweat test were reviewed. Pattern and lesion site (preganglionic, postganglionic, or mixed) were determined based on thermoregulatory sweat test and postganglionic sudomotor testing...
November 10, 2016: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/27848084/default-mode-network-connectivity-in-cognitively-unimpaired-drug-na%C3%A3-ve-patients-with-rigidity-dominant-parkinson-s-disease
#8
Yanbing Hou, Chunyan Luo, Jing Yang, Ruwei Ou, Wanglin Liu, Wei Song, Qiyong Gong, Huifang Shang
Parkinson's disease (PD) with akinetic rigidity (PDAR) is more likely to develop cognitive deficits compared to PD with tremor-dominant symptoms (PDTD). The default mode network (DMN) is highly relevant for cognitive processes, so this study tested the functional connectivity (FC) of DMN in cognitively unimpaired PDAR patients. Resting-state fMRI data were collected in 21 cognitively unimpaired early stage drug-naïve patients with PDAR and 21 healthy controls (HC). PD patients were matched closely to HCs for demographic and cognitive variables...
November 15, 2016: Journal of Neurology
https://www.readbyqxmd.com/read/27844341/does-the-type-of-multisystem-atrophy-parkinsonism-or-cerebellar-ataxia-impact-on-the-nature-of-sleep-disorders
#9
REVIEW
Luigi Ferini-Strambi, Sara Marelli, Romina Combi
Multiple system atrophy (MSA) is a neurodegenerative disease characterized by a combination of autonomic failure, parkinsonism, and/or cerebellar ataxia. The cause of MSA is unknown, but neuropathologically the disease is characterized by widespread α-synuclein-positive glial cytoplasmic inclusions and striatonigral and/or olivopontocerebellar neurodegeneration. Two motor phenotypes have been clinically identified: parkinsonian (MSA-P) and cerebellar (MSA-C). In order to elucidate if in addition to the motor abnormalities there are other significant differences between these two phenotypes, we performed a review of the studies on sleep disorders in the two MSA subtypes...
December 2016: Current Neurology and Neuroscience Reports
https://www.readbyqxmd.com/read/27837368/comparison-of-neutrophil-lymphocyte-ratio-nlr-in-parkinson-s-disease-subtypes
#10
Ceyla Ataç Uçar, Burcu Gökçe Çokal, Hanzade Aybüke Ünal Artık, Levent Ertuğrul İnan, Tahir Kurtuluş Yoldaş
It has been hypothesized that chronic inflammation may play an important role in the development and progression of the neurodegeneration of Idiopathic Parkinson's disease (IPD). Neutrophil-lymphocyte ratio (NLR) is a marker that indicates the peripheral inflammation. There is only one study regarding NLR and IPD. In this study, we assessed to investigate NLR in patients with IPD, comparing the results with controls and to determine whether there is a difference in NLR levels in subgroups of IPD (akinetic-rigid and tremor-dominant) differ in their levels of NLR...
November 11, 2016: Neurological Sciences
https://www.readbyqxmd.com/read/27826740/calcium-channel-antagonists-as-disease-modifying-therapy-for-parkinson-s-disease-therapeutic-rationale-and-current-status
#11
Tara Swart, Michael J Hurley
Parkinson's disease is a disabling hypokinetic neurological movement disorder in which the aetiology is unknown in the majority of cases. Current pharmacological treatments, though effective at restoring movement, are only symptomatic and do nothing to slow disease progression. Electrophysiological, epidemiological and neuropathological studies have implicated CaV1.3 subtype calcium channels in the pathogenesis of the disorder, and drugs with some selectivity for this ion channel (brain-penetrant dihydropyridine calcium channel blockers) are neuroprotective in animal models of the disease...
November 8, 2016: CNS Drugs
https://www.readbyqxmd.com/read/27812030/predicting-subtype-selectivity-for-adenosine-receptor-ligands-with-three-dimensional-biologically-relevant-spectrum-brs-3d
#12
Song-Bing He, Ben Hu, Zheng-Kun Kuang, Dong Wang, De-Xin Kong
Adenosine receptors (ARs) are potential therapeutic targets for Parkinson's disease, diabetes, pain, stroke and cancers. Prediction of subtype selectivity is therefore important from both therapeutic and mechanistic perspectives. In this paper, we introduced a shape similarity profile as molecular descriptor, namely three-dimensional biologically relevant spectrum (BRS-3D), for AR selectivity prediction. Pairwise regression and discrimination models were built with the support vector machine methods. The average determination coefficient (r(2)) of the regression models was 0...
November 4, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27785931/role-of-glutathione-s-transferases-in-neurological-problems
#13
Anil Kumar, Dinesh K Dhull, Varun Gupta, Priyanka Channana, Arti Singh, Manveen Bhardwaj, Poonam Ruhal, Ruchika Mittal
Role of Glutathione-S-transferases (GSTs) has been well explored in the cellular detoxification process, regulation of redox homeostasis and S-glutothionylation of target proteins like JNK, ASK1 etc. However, altered levels or functions of this enzyme or their subtypes have emerged in the development of several pathologies diseases such as Alzheimer's disease, Parkinson's disease, cancer and related conditions. Oxidative stress is one of the possible pathological events that contributes significantly to activation of degenerating cascades inside neuronal cells...
October 27, 2016: Expert Opinion on Therapeutic Patents
https://www.readbyqxmd.com/read/27777423/characterization-and-transplantation-of-enteric-neural-crest-cells-from-human-induced-pluripotent-stem-cells
#14
W Li, L Huang, J Zeng, W Lin, K Li, J Sun, W Huang, J Chen, G Wang, Q Ke, J Duan, X Lai, R Chen, M Liu, Y Liu, T Wang, X Yang, Y Chen, H Xia, A P Xiang
The enteric nervous system (ENS) is recognized as a second brain because of its complexity and its largely autonomic control of bowel function. Recent progress in studying the interactions between the ENS and the central nervous system (CNS) has implicated alterations of the gut/brain axis as a possible mechanism in the pathophysiology of autism spectrum disorders (ASDs), Parkinson's disease (PD) and other human CNS disorders, whereas the underlying mechanisms are largely unknown because of the lack of good model systems...
October 25, 2016: Molecular Psychiatry
https://www.readbyqxmd.com/read/27772736/vitamin-d-receptor-gene-polymorphisms-and-cognitive-decline-in-parkinson-s-disease
#15
Nicole M Gatto, Kimberly C Paul, Janet S Sinsheimer, Jeff M Bronstein, Yvette Bordelon, Rebecca Rausch, Beate Ritz
We and others have suggested that vitamin D receptor gene (VDR) polymorphisms influence susceptibility for Parkinson's disease (PD), Alzheimer's disease (AD), mild cognitive impairment (MCI) or overall cognitive functioning. Here we examine VDR polymorphisms and cognitive decline in patients with PD. Non-Hispanic Caucasian PD patients (n=190) in the Parkinson Environment Gene (PEG) study were successfully genotyped for seven VDR polymorphisms. Cognitive function was assessed with the Mini-Mental State Exam (MMSE) at baseline and at a maximum of three follow-up exams...
November 15, 2016: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/27756506/transforming-growth-factor-%C3%AE-1-in-the-cerebrospinal-fluid-of-patients-with-distinct-neurodegenerative-diseases
#16
Tomoyuki Masuda, Junko Itoh, Takuya Koide, Yasushi Tomidokoro, Yosuke Takei, Kazuhiro Ishii, Akira Tamaoka
A chronic inflammatory condition may underlie neurodegenerative disorders, including Parkinson's disease (PD) and Alzheimer's disease (AD). For example, both PD and AD patients show an increase in transforming growth factor-β1 (TGF-β1) levels in their cerebrospinal fluid (CSF). TGF-β1 is a cytokine that inhibits inflammation. In the present study, using an enzyme-linked immunosorbent assay, we tested the hypothesis that the level of TGF-β1 in the CSF of patients with amyotrophic lateral sclerosis (ALS), spinocerebellar degeneration (SCD), or multiple system atrophy-cerebellar subtype (MSA-C) would be elevated compared with that of normal controls...
October 15, 2016: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/27753188/clinical-and-demographic-characteristics-related-to-onset-site-and-spread-of-cervical-dystonia
#17
Scott A Norris, H A Jinnah, Alberto J Espay, Christine Klein, Norbert Brüggemann, Richard L Barbano, Irene Andonia C Malaty, Ramon L Rodriguez, Marie Vidailhet, Emmanuel Roze, Stephen G Reich, Brian D Berman, Mark S LeDoux, Sarah Pirio Richardson, Pinky Agarwal, Zoltan Mari, William G Ondo, Ludy C Shih, Susan H Fox, Alfredo Berardelli, Claudia M Testa, Florence Ching-Fen Cheng, Daniel Truong, Fatta B Nahab, Tao Xie, Mark Hallett, Ami R Rosen, Laura J Wright, Joel S Perlmutter
BACKGROUND: Clinical characteristics of isolated idiopathic cervical dystonia such as onset site and spread to and from additional body regions have been addressed in single-site studies with limited data and incomplete or variable dissociation of focal and segmental subtypes. The objectives of this study were to characterize the clinical characteristics and demographics of isolated idiopathic cervical dystonia in the largest standardized multicenter cohort. METHODS: The Dystonia Coalition, through a consortium of 37 recruiting sites in North America, Europe, and Australia, recruited 1477 participants with focal (60...
October 18, 2016: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/27749396/clinical-subtypes-and-genetic-heterogeneity-of-lumping-and-splitting-in-parkinson-disease
#18
Rainer von Coelln, Lisa M Shulman
PURPOSE OF REVIEW: Recent studies on clinical, genetic and pathological heterogeneity of Parkinson disease have renewed the old debate whether we should think of Parkinson disease as one disease with variations, or as a group of independent diseases that happen to present with similar phenotypes. Here, we provide an overview of where the debate is coming from, and how recent findings in clinical subtyping, genetics and clinico-pathological correlation have shaped this controversy over the last few years...
December 2016: Current Opinion in Neurology
https://www.readbyqxmd.com/read/27720902/targeted-differentiation-of-regional-ventral-neuroprogenitors-and-related-neuronal-subtypes-from-human-pluripotent-stem-cells
#19
Liankai Chi, Beibei Fan, Kunshan Zhang, Yanhua Du, Zhongliang Liu, Yujiang Fang, Zhenyu Chen, Xudong Ren, Xiangjie Xu, Cizhong Jiang, Siguang Li, Lin Ma, Liang Gao, Ling Liu, Xiaoqing Zhang
Embryoid body (EB) formation and adherent culture (AD) paradigms are equivalently thought to be applicable for neural specification of human pluripotent stem cells. Here, we report that sonic hedgehog-induced ventral neuroprogenitors under EB conditions are fated to medial ganglionic eminence (MGE), while the AD cells mostly adopt a floor-plate (FP) fate. The EB-MGE later on differentiates into GABA and cholinergic neurons, while the AD-FP favors dopaminergic neuron specification. Distinct developmental, metabolic, and adhesion traits in AD and EB cells may potentially account for their differential patterning potency...
November 8, 2016: Stem Cell Reports
https://www.readbyqxmd.com/read/27719845/psychogenic-functional-parkinsonism
#20
M A Thenganatt, J Jankovic
Psychogenic parkinsonism (PP), although often quite disabling, is one of the least commonly reported subtypes of psychogenic movement disorders. There are certain features that help distinguish PP from idiopathic Parkinson's disease, such as abrupt onset, early disability, bilateral shaking and slowness, nondecremental slowness when performing repetitive movements, voluntary resistance against passive movement without cogwheel rigidity, distractibility, "give-way" weakness, stuttering speech, bizarre gait, and a variety of behavioral symptoms...
2017: Handbook of Clinical Neurology
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