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https://www.readbyqxmd.com/read/29150919/a-focus-on-the-beneficial-effects-of-alpha-synuclein-and-a-re-appraisal-of-synucleinopathies
#1
Larisa Ryskalin, Carla L Busceti, Fiona Limanaqi, Francesca Biagioni, Stefano Gambardella, Francesco Fornai
Alpha synuclein (α-syn) belongs to a class of proteins which are commonly considered to play a detrimental role in neuronal survival. This assumption is based on the occurrence of a severe neuronal degeneration in patients carrying a multiplication of the α-syn gene (SNCA) and in a variety of experimental models, where overexpression of α-syn leads to cell death and neurological impairment. In these conditions, a higher amount of normally structured α-syn produces a damage, which is even worse compared with that produced by α-syn owing an abnormal structure (as occurring following point gene mutations)...
November 16, 2017: Current Protein & Peptide Science
https://www.readbyqxmd.com/read/29150334/a-routinely-used-protein-staining-dye-acts-as-an-inhibitor-of-wild-type-and-mutant-alpha-synuclein-aggregation-and-modulator-of-neurotoxicity
#2
Nuzhat Ahsan, Ibrar Ahmed Siddique, Sarika Gupta, Avadhesha Surolia
Inhibition of amyloid formation along with modulation of toxicity employing small molecules is emerging as a potential therapeutic approach for protein misfolding disorders which includes Parkinson's disease, Alzheimer's disease and Multiple System Atrophy etc. Countless current interventional strategies for treating α-synucleinopathies consider using peptidic and non-peptidic inhibitors for arresting fibrillisation, disrupting existing fibrils and reducing associated toxicity. One group of molecules less exploited in this regard are triphenylmethane dyes...
October 12, 2017: European Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/29146111/microrna-expression-patterns-in-human-anterior-cingulate-and-motor-cortex-a-study-of-dementia-with-lewy-bodies-cases-and-controls
#3
Peter T Nelson, Wang-Xia Wang, Sarah A Janse, Katherine L Thompson
OVERVIEW: MicroRNAs (miRNAs) have been implicated in neurodegenerative diseases including Parkinson's disease and Alzheimer's disease (AD). Here, we evaluated the expression of miRNAs in anterior cingulate (AC; Brodmann area [BA] 24) and primary motor (MO; BA 4) cortical tissue from aged human brains in the University of Kentucky AD Center autopsy cohort, with a focus on dementia with Lewy bodies (DLB). METHODS: RNA was isolated from gray matter of brain samples with pathology-defined DLB, AD, AD+DLB, and low-pathology controls, with n=52 cases initially included (n=23 with DLB), all with low (<4hrs) postmortem intervals...
November 13, 2017: Brain Research
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/29124503/urological-dysfunction-in-synucleinopathies-epidemiology-pathophysiology-and-management
#5
REVIEW
Ryuji Sakakibara, Fuyuki Tateno, Tatsuya Yamamoto, Tomoyuki Uchiyama, Tomonori Yamanishi
OBJECTIVE: Parkinson's disease (PD) and multiple system atrophy (MSA) are major neurogenerative diseases characterized pathologically by abnormal alpha-synuclein aggregation. PD and MSA are clinically characterized by motor disorder and bladder dysfunction (mainly urinary urgency and frequency, also called overactive bladder). However, few literatures are available concerning bladder dysfunction in PD or MSA. METHOD: A systematic review. RESULTS: The bladder dysfunction in MSA is more severe than that in PD for large post-void residual or urinary retention...
November 9, 2017: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
https://www.readbyqxmd.com/read/29119326/neurodegeneration-and-the-ordered-assembly-of-%C3%AE-synuclein
#6
REVIEW
Maria Grazia Spillantini, Michel Goedert
In 2017, it was 200 years since James Parkinson published 'An Essay on the Shaking Palsy' and 20 years since α-synuclein aggregation came to the fore. In 1998, multiple system atrophy joined Parkinson's disease and dementia with Lewy bodies as the third major synucleinopathy. Here, we describe the work that led to the identification of α-synuclein in Lewy bodies, Lewy neurites and Papp-Lantos bodies. We also review some of the findings reported since 1997.
November 8, 2017: Cell and Tissue Research
https://www.readbyqxmd.com/read/29116402/a-critical-review-of-the-prion-hypothesis-of-human-synucleinopathies
#7
REVIEW
Gültekin Tamgüney, Amos D Korczyn
Parkinson's disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA) are neurodegenerative disorders which have been pathologically classified as synucleinopathies, since they are associated with pathognomonic deposits of misfolded alpha-synuclein in cells of the nervous system. Recently PD, DLB, and MSA were also suggested to be prion-like disorders. Much controversy exists regarding this analogy between synucleinopathies and prion diseases. Here, we discuss what characterizes prion diseases and in which way synucleinopathies may be considered prion-like or -unlike...
November 8, 2017: Cell and Tissue Research
https://www.readbyqxmd.com/read/29110782/biomarkers-in-cerebrospinal-fluid-for-synucleinopathies-tauopathies-and-other-neurodegenerative-disorders
#8
Tainá M Marques, Anouke Van Rumund, H Bea Kuiperij, Marcel M Verbeek
The incidence of neurodegenerative disorders is increasing due to worldwide population aging. In general, sporadic forms account for 90% of total cases with neurodegenerative disorders and the reasons underlying initiation or progression of these diseases remain unknown for almost all disorders. To date, diagnosis is mainly based on clinical symptoms and neuroimaging, which is in many cases insufficient due to overlap in clinical symptoms among several neurodegenerative disorders. Therefore, postmortem neuropathologic confirmation remains the gold-standard diagnostic technique for many disorders...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29103051/c-abl-and-parkinson-s-disease-mechanisms-and-therapeutic-potential
#9
Saurav Brahmachari, Senthilkumar S Karuppagounder, Preston Ge, Saebom Lee, Valina L Dawson, Ted M Dawson, Han Seok Ko
 Although the etiology of Parkinson's disease (PD) is poorly understood, oxidative stress has long been implicated in the pathogenesis of the disease. However, multifaceted and divergent signaling cascades downstream of oxidative stress have posed challenges for researchers to identify a central component of the oxidative stress-induced pathways causing neurodegeneration in PD. Since 2010, c-Abl-a non-receptor tyrosine kinase and an indicator of oxidative stress-has shown remarkable potential as a future promising drug target in PD therapeutics...
2017: Journal of Parkinson's Disease
https://www.readbyqxmd.com/read/29101940/rem-sleep-behavior-disorder-diagnosis-clinical-implications-and-future-directions
#10
REVIEW
Erik K St Louis, Bradley F Boeve
Rapid eye movement sleep behavior disorder (RBD) is diagnosed by a clinical history of dream enactment accompanied by polysomnographic rapid eye movement sleep atonia loss (rapid eye movement sleep without atonia). Rapid eye movement sleep behavior disorder is strongly associated with neurodegenerative disease, especially synucleinopathies such as Parkinson disease, dementia with Lewy bodies, and multiple system atrophy. A history of RBD may begin several years to decades before onset of any clear daytime symptoms of motor, cognitive, or autonomic impairments, suggesting that RBD is the presenting manifestation of a neurodegenerative process...
November 2017: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/29092095/a%C3%AE-exacerbates-%C3%AE-synuclein-induced-neurotoxicity-through-impaired-insulin-signaling-in-%C3%AE-synuclein-overexpressed-human-sk-n-mc-neuronal-cells
#11
Ching-Chi Chang, Hsin-Hua Li, Yen-Ting Chang, Ying-Jui Ho, Ling-Jia Hsieh, Pai-Yi Chiu, Yu-Shih Cheng, Chih-Li Lin, Te-Jen Lai
AIM: α-Synuclein (αSyn) is known as a small soluble protein abundantly expressed in neuronal cells. Although its physiological role is still unclear, the aggregation of αSyn has been recognized as responsible for some neurodegenerative disorders such as dementia with Lewy bodies (DLB). In most cases, intracellular abnormal aggregates are caused by protein-coding mutations that alter primary structure and therefore increase propensity toward aggregation. However, no pathogenic alterations or polymorphisms in αSyn are found in DLB patients so far, suggesting genetic mutations may not play a major role in DLB pathogenesis...
November 1, 2017: CNS Neuroscience & Therapeutics
https://www.readbyqxmd.com/read/29089869/microrna-101-modulates-autophagy-and-oligodendroglial-alpha-synuclein-accumulation-in-multiple-system-atrophy
#12
Elvira Valera, Brian Spencer, Jennifer Mott, Margarita Trejo, Anthony Adame, Michael Mante, Edward Rockenstein, Juan C Troncoso, Thomas G Beach, Eliezer Masliah, Paula Desplats
Synucleinopathies, neurodegenerative disorders with alpha-synuclein (α-syn) accumulation, are the second leading cause of neurodegeneration in the elderly, however no effective disease-modifying alternatives exist for these diseases. Multiple system atrophy (MSA) is a fatal synucleinopathy characterized by the accumulation of toxic aggregates of α-syn within oligodendroglial cells, leading to demyelination and neurodegeneration, and the reduction of this accumulation might halt the fast progression of MSA...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/29064079/molecular-mechanisms-of-%C3%AE-synuclein-and-gba1-in-parkinson-s-disease
#13
REVIEW
Iva Stojkovska, Dimitri Krainc, Joseph R Mazzulli
Parkinson's disease (PD) is a neurodegenerative movement disorder characterized pathologically by the presence of Lewy bodies comprised of insoluble alpha (α)-synuclein. Pathological, clinical and genetic studies demonstrate that mutations in the GBA1 gene, which encodes the lysosomal enzyme glucocerebrosidase (GCase) that is deficient in Gaucher's disease, are important risk factors for the development of PD. The molecular mechanism for the association between these two diseases is not completely understood...
October 24, 2017: Cell and Tissue Research
https://www.readbyqxmd.com/read/29063640/amyotrophic-lateral-sclerosis-and-parkinsonism-dementia-complex-of-the-hohara-focus-of-the-kii-peninsula-a-multiple-proteinopathy
#14
REVIEW
Maya Mimuro, Mari Yoshida, Shigeki Kuzuhara, Yasumasa Kokubo
The high incidence of amyotrophic lateral sclerosis (ALS) and parkinsonism-dementia complex (PDC) has been previously known in the Kii Peninsula of Japan and in Guam. Recently, the accumulation of various proteins, such as tau, trans-activation response DNA binding protein 43 kDa (TDP-43), and alpha-synuclein (αSyn), was reported in the brains of patients with ALS/PDC in Guam. To confirm whether similar findings are present in Kii ALS/PDC, we neuropathologically examined the brains and spinal cords of 18 patients with ALS/PDC (clinical diagnoses: eight ALS and 10 PDC) in Hohara Village, which is the eastern focus of Kii ALS...
October 23, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29058121/multiple-system-atrophy-experimental-models-and-reality
#15
REVIEW
Cassia Overk, Edward Rockenstein, Elvira Valera, Nadia Stefanova, Gregor Wenning, Eliezer Masliah
Multiple system atrophy (MSA) is a rapidly progressing fatal synucleinopathy of the aging population characterized by parkinsonism, dysautonomia, and in some cases ataxia. Unlike other synucleinopathies, in this disorder the synaptic protein, α-synuclein (α-syn), predominantly accumulates in oligodendroglial cells (and to some extent in neurons), leading to maturation defects of oligodendrocytes, demyelination, and neurodegeneration. The mechanisms through which α-syn deposits occur in oligodendrocytes and neurons in MSA are not completely clear...
October 20, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/29056362/critical-appraisal-of-pathology-transmission-in-the-%C3%AE-synuclein-fibril-model-of-lewy-body-disorders
#16
Negin Nouraei, Daniel M Mason, Kristin M Miner, Michael A Carcella, Tarun N Bhatia, Benjamin K Dumm, Dishaben Soni, David A Johnson, Kelvin C Luk, Rehana K Leak
Lewy body disorders are characterized by the emergence of α-synucleinopathy in many parts of the central and peripheral nervous systems, including in the telencephalon. Dense α-synuclein(+) pathology appears in regio inferior of the hippocampus in both Parkinson's disease and dementia with Lewy bodies and may disturb cognitive function. The preformed α-synuclein fibril model of Parkinson's disease is growing in use, given its potential for seeding the self-propagating spread of α-synucleinopathy throughout the mammalian brain...
October 19, 2017: Experimental Neurology
https://www.readbyqxmd.com/read/29048372/%C3%AE-synuclein-regulates-neuronal-cholesterol-efflux
#17
Jen-Hsiang T Hsiao, Glenda M Halliday, Woojin Scott Kim
α-Synuclein is a neuronal protein that is at the center of focus in understanding the etiology of a group of neurodegenerative diseases called α-synucleinopathies, which includes Parkinson's disease (PD). Despite much research, the exact physiological function of α-synuclein is still unclear. α-Synuclein has similar biophysical properties as apolipoproteins and other lipid-binding proteins and has a high affinity for cholesterol. These properties suggest a possible role for α-synuclein as a lipid acceptor mediating cholesterol efflux (the process of removing cholesterol out of cells)...
October 19, 2017: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
https://www.readbyqxmd.com/read/29046661/mitophagy-in-parkinson-s-disease-pathogenic-and-therapeutic-implications
#18
REVIEW
Fei Gao, Jia Yang, Dongdong Wang, Chao Li, Yi Fu, Huaishan Wang, Wei He, Jianmin Zhang
Neurons affected in Parkinson's disease (PD) experience mitochondrial dysfunction and bioenergetic deficits that occur early and promote the disease-related α-synucleinopathy. Emerging findings suggest that the autophagy-lysosome pathway, which removes damaged mitochondria (mitophagy), is also compromised in PD and results in the accumulation of dysfunctional mitochondria. Studies using genetic-modulated or toxin-induced animal and cellular models as well as postmortem human tissue indicate that impaired mitophagy might be a critical factor in the pathogenesis of synaptic dysfunction and the aggregation of misfolded proteins, which in turn impairs mitochondrial homeostasis...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/29040593/oral-exposure-to-paraquat-triggers-earlier-expression-of-phosphorylated-%C3%AE-synuclein-in-the-enteric-nervous-system-of-a53t-mutant-human-%C3%AE-synuclein-transgenic-mice
#19
Nicolas Naudet, Emilie Antier, Damien Gaillard, Eric Morignat, Latifa Lakhdar, Thierry Baron, Anna Bencsik
The misfolded α-synuclein protein, phosphorylated at serine 129 (pSer129 α-syn), is the hallmark of Parkinson disease (PD). Detected also in the enteric nervous system (ENS), it supports the recent theory that PD could start in the gut, rather than the brain. In a previous study, using a transgenic mouse model of human synucleinopathies expressing the A53T mutant α-synuclein (TgM83), in which a neurodegenerative process associated with α-synuclein occurs spontaneously in the brain, we have shown earlier onset of pSer129 α-syn in the ENS...
December 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/29036344/comparison-of-the-in-vivo-induction-and-transmission-of-%C3%AE-synuclein-pathology-by-mutant-%C3%AE-synuclein-fibril-seeds-in-transgenic-mice
#20
Nicola J Rutherford, Jess-Karan S Dhillon, Cara J Riffe, Jasie K Howard, Mieu Brooks, Benoit I Giasson
Parkinson's disease is one of many neurodegenerative diseases termed synucleinopathies, neuropathologically defined by inclusions containing aggregated α-synuclein. α-synuclein gene (SNCA) mutations can directly cause autosomal dominant Parkinson's disease. In vitro studies demonstrated that SNCA missense mutations may either enhance or diminish α-synuclein aggregation but cross seeding of mutant and wild-type α-synuclein proteins appear to reduce aggregation efficiency. Here we extended these studies by assessing the effects of seeded α-synuclein aggregation in α-synuclein transgenic mice through intracerebral or peripheral injection of various mutant α-synuclein fibrils...
September 27, 2017: Human Molecular Genetics
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