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Neurodegenerative diseases

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https://www.readbyqxmd.com/read/28545157/the-aminoestrogen-prolame-increases-recognition-memory-and-hippocampal-neuronal-spine-density-in-aged-mice
#1
Alfonso Diaz, Samuel Treviño, Rubén Vázquez-Roque, Berenice Venegas, Blanca Espinosa, Gonzalo Flores, Juan Manuel Fernández-G, Luis F Montaño, Jorge Guevara
The aging brain shows biochemical and morphological changes in the dendrites of pyramidal neurons from the limbic system associated with memory loss. Prolame (N-(3-hydroxy-1,3,5 (10) -estratrien-17β-yl) -3-hydroxypropylamine) is a non-feminizing aminoestrogen with antithrombotic activity that prevents neuronal deterioration, oxidative stress and neuroinflammation. Our aim was to evaluate the effect of prolame on motor and cognitive processes, as well as its influence on the dendritic morphology of neurons at the CA1, CA3 and granule cells of the dentate gyrus (DG) regions of hippocampus (HP), and medium spiny neurons of the nucleus accumbens (NAcc) of aged mice...
May 25, 2017: Synapse
https://www.readbyqxmd.com/read/28545141/prion-pathogenesis-is-unaltered-in-the-absence-of-sirp%C3%AE-mediated-don-t-eat-me-signaling
#2
Mario Nuvolone, Marta Paolucci, Silvia Sorce, Veronika Kana, Rita Moos, Takashi Matozaki, Adriano Aguzzi
Prion diseases are neurodegenerative conditions caused by misfolding of the prion protein, leading to conspicuous neuronal loss and intense microgliosis. Recent experimental evidence point towards a protective role of microglia against prion-induced neurodegeneration, possibly through elimination of prion-containing apoptotic bodies. The molecular mechanisms by which microglia recognize and eliminate apoptotic cells in the context of prion diseases are poorly defined. Here we investigated the possible involvement of signal regulatory protein α (SIRPα), a key modulator of host cell phagocytosis; SIRPα is encoded by the Sirpa gene that is genetically linked to the prion gene Prnp...
2017: PloS One
https://www.readbyqxmd.com/read/28544479/drinking-problems-mechanisms-of-macropinosome-formation-and-maturation
#3
REVIEW
Catherine M Buckley, Jason S King
Macropinocytosis is a mechanism for the non-specific bulk uptake and internalisation of extracellular fluid. This plays specific and distinct roles in diverse cell types such as macrophages, dendritic cells and neurons, by allowing cells to sample their environment, extract extracellular nutrients and regulate plasma membrane turnover. Macropinocytosis has recently been implicated in several diseases including cancer, neurodegenerative diseases and atherosclerosis. Uptake by macropinocytosis is also exploited by several intracellular pathogens to gain entry into host cells...
May 24, 2017: FEBS Journal
https://www.readbyqxmd.com/read/28544038/supplementation-with-herbal-extracts-to-promote-behavioral-and-neuroprotective-effects-in-experimental-models-of-parkinson-s-disease-a-systematic-review
#4
REVIEW
Ianara Mendonça da Costa, José Rodolfo Lopes de Paiva Cavalcanti, Dinalva Brito de Queiroz, Eduardo Pereira de Azevedo, Amália Cinthia Meneses do Rêgo, Irami Araújo Filho, Paulo Parente, Marco Antônio Botelho, Fausto Pierdoná Guzen
Parkinson's disease (PD) consists of a neurodegenerative pathology that has received a considerable amount of attention because of its clinical manifestations. The most common treatment consists of administering the drugs levodopa and biperiden, which reduce the effectiveness of the disease and the progress of its symptoms. However, phytotherapy treatment of PD has shown great potential in retarding the loss of dopaminergic neurons and minimizing the behavioral abnormalities. The aim of this study is to systematically review the use of supplemental herbal plants with cellular protective effect and behavioral activity in in vivo and in vitro experimental models...
May 22, 2017: Phytotherapy Research: PTR
https://www.readbyqxmd.com/read/28543935/neurodegeneration-in-ataxia-telangiectasia-multiple-roles-of-atm-kinase-in-cellular-homeostasis
#5
REVIEW
Kay Rui Choy, Dianne J Watters
Ataxia-Telangiectasia (A-T) is characterized by neuronal degeneration, cancer, diabetes, immune deficiency and increased sensitivity to ionizing radiation. A-T is attributed to the deficiency of the protein kinase coded by the ATM (Ataxia-Telangiectasia Mutated) gene. ATM is a sensor of DNA Double Strand Breaks and signals to cell cycle checkpoints and the DNA repair machinery. ATM phosphorylates numerous substrates and activates many cell-signalling pathways. There has been considerable debate about whether a defective DNA damage response is causative of the neurological aspects of the disease...
May 22, 2017: Developmental Dynamics: An Official Publication of the American Association of Anatomists
https://www.readbyqxmd.com/read/28543594/the-neuroprotective-role-of-mir-124-3p-in-a-6-hydroxydopamine-induced-cell-model-of-parkinson-s-disease-via-the-regulation-of-anax5
#6
Rui-Fang Dong, Bing Zhang, Li-Wen Tai, Hong-Mei Liu, Fang-Kun Shi, Ning-Ning Liu
Parkinson's disease (PD), the second most common neurodegenerative disorder, is characterized by a progressive loss of dopaminergic neurons in the midbrain. Several pathogenetic factors have been involved in the onset and progression of PD, including inflammation, oxidative stress, unfolded protein accumulation, and apoptosis. Ample evidence indicates that miRNAs could regulate post-transcriptional gene expression and neuronal disease. In this study, we evaluated the effects and mechanism of miR-124-3p on 6-hydroxydopamine (6-OHDA)-induced neurotoxicity in PC12 cells and SH-SY5Y cells...
May 25, 2017: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/28543180/traf6-participates-in-early-brain-injury-after-subarachnoid-hemorrhage-in-rats-through-inhibiting-autophagy-and-promoting-oxidative-stress
#7
Yang Dou, Haitao Shen, Dongxia Feng, Haiying Li, Xiaodi Tian, Jian Zhang, Zhong Wang, Gang Chen
Tumor necrosis factor receptor-associated factor 6 (TRAF6) is a member of the TRAF family and an important multifunctional intracellular adaptin of the tumor necrosis factor (TNF) superfamily and toll/IL-1 receptor (TIR) superfamily. TRAF6 has been studied in several central nervous system (CNS) diseases, including ischemic stroke, traumatic brain injury and neurodegenerative diseases, but its role in subarachnoid hemorrhage (SAH) has not been fully illustrated. This study was designed to explore changes of expression level and potential roles and mechanisms of TRAF6 in early brain injury (EBI) after SAH by using a Sprague-Dawley rat model of SAH induced in 0...
May 24, 2017: Journal of Neurochemistry
https://www.readbyqxmd.com/read/28543166/chronic-emgs-in-treadmill-running-sod1-mice-reveal-early-changes-in-muscle-activation
#8
K A Quinlan, E Kajtaz, J D Ciolino, R D Imhoff-Manuel, M C Tresch, C J Heckman, V M Tysseling
To improve our understanding of early disease mechanisms and find reliable biomarkers of amyotrophic lateral sclerosis (ALS), a progressive neurodegenerative disease, we measured electromyogram (EMG) activity in hind limb muscles of SOD1G93A mice. In contrast to clinical diagnostic measures using EMGs, which are performed on quiescent patients, we monitored activity during treadmill running in order to detect presymptomatic changes in motor patterning. Chronic EMG electrodes were implanted into vastus lateralis (VL), biceps femoris posterior (BFP), lateral gastrocnemius (LG), and tibialis anterior (TA) in mice from postnatal day (P) 55-100, and results were assessed using linear mixed models...
May 24, 2017: Journal of Physiology
https://www.readbyqxmd.com/read/28542792/on-the-complexity-of-clinical-and-molecular-bases-of-neurodegeneration-with-brain-iron-accumulation
#9
REVIEW
Cristina Tello, Alejandra Darling, Vincenzo Lupo, Belén Pérez-Dueñas, Carmen Espinós
Neurodegeneration with brain iron accumulation (NBIA) are a group of inherited heterogeneous neurodegenerative rare disorders. These patients present with dystonia, spasticity, parkinsonism and neuropsychiatric disturbances, along with brain MRI evidence of iron accumulation. In sum, they are devastating disorders and to date, there is no specific treatment. Ten NBIA genes are accepted: PANK2, PLA2G6, C19orf12, COASY, FA2H, ATP13A2, WDR45, FTL, CP, and DCAF17, and. Nonetheless, a relevant percentage of patients remain without genetic diagnosis, suggesting that other novel NBIA genes remain to be discovered...
May 23, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28542676/detailed-clinical-phenotype-and-molecular-genetic-findings-in-cln3-associated-isolated-retinal-degeneration
#10
Cristy A Ku, Sarah Hull, Gavin Arno, Ajoy Vincent, Keren Carss, Robert Kayton, Douglas Weeks, Glenn W Anderson, Ryan Geraets, Camille Parker, David A Pearce, Michel Michaelides, Robert E MacLaren, Anthony G Robson, Graham E Holder, Elise Heon, F Lucy Raymond, Anthony T Moore, Andrew R Webster, Mark E Pennesi
Importance: Mutations in genes traditionally associated with syndromic retinal disease are increasingly found to cause nonsyndromic inherited retinal degenerations. Mutations in CLN3 are classically associated with juvenile neuronal ceroid lipofuscinosis, a rare neurodegenerative disease with early retinal degeneration and progressive neurologic deterioration, but have recently also been identified in patients with nonsyndromic inherited retinal degenerations. To our knowledge, detailed clinical characterization of such cases has yet to be reported...
May 25, 2017: JAMA Ophthalmology
https://www.readbyqxmd.com/read/28542401/molecular-dynamics-analysis-of-the-aggregation-propensity-of-polyglutamine-segments
#11
Jingran Wen, Daniel R Scoles, Julio C Facelli
Protein misfolding and aggregation is a pathogenic feature shared among at least ten polyglutamine (polyQ) neurodegenerative diseases. While solvent-solution interaction is a key factor driving protein folding and aggregation, the solvation properties of expanded polyQ tracts are not well understood. By using GPU-enabled all-atom molecular dynamics simulations of polyQ monomers in an explicit solvent environment, this study shows that solvent-polyQ interaction propensity decreases as the lengths of polyQ tract increases...
2017: PloS One
https://www.readbyqxmd.com/read/28542381/quantitative-magnetic-resonance-imaging-of-brain-atrophy-in-a-mouse-model-of-niemann-pick-type-c-disease
#12
John W Totenhagen, Adam Bernstein, Eriko S Yoshimaru, Robert P Erickson, Theodore P Trouard
In vivo magnetic resonance imaging (MRI) was used to investigate regional and global brain atrophy in the neurodegenerative Niemann Pick Type C1 (NPC1) disease mouse model. Imaging experiments were conducted with the most commonly studied mouse model of NPC1 disease at early and late disease states. High-resolution in vivo images were acquired at early and late stages of the disease and analyzed with atlas-based registration to obtain measurements of twenty brain region volumes. A two-way ANOVA analysis indicated eighteen of these regions were different due to genotype and thirteen showed a significant interaction with age and genotype...
2017: PloS One
https://www.readbyqxmd.com/read/28542144/dicer-and-micrornas-protect-adult-dopamine-neurons
#13
Piotr Chmielarz, Julia Konovalova, Syeda Sadia Najam, Heike Alter, Timo Petteri Piepponen, Holger Erfle, Kai C Sonntag, Günther Schütz, Ilya A Vinnikov, Andrii Domanskyi
MicroRNAs (miRs) are important post-transcriptional regulators of gene expression implicated in neuronal development, differentiation, aging and neurodegenerative diseases, including Parkinson's disease (PD). Several miRs have been linked to PD-associated genes, apoptosis and stress response pathways, suggesting that deregulation of miRs may contribute to the development of the neurodegenerative phenotype. Here, we investigate the cell-autonomous role of miR processing RNAse Dicer in the functional maintenance of adult dopamine (DA) neurons...
May 25, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28542141/an-enhanced-bioluminescence-based-annexin-v-probe-for-apoptosis-detection-in-vitro-and-in-vivo
#14
Trajen Head, Peter Dau, Stephanie Duffort, Pirouz Daftarian, Pratibha M Joshi, Roberto Vazquez-Padron, Sapna K Deo, Sylvia Daunert
The process of controlled cellular death known as apoptosis has an important central role not only in normal homeostatic maintenance of tissues, but also in numerous diseases such as cancer, neurodegenerative, autoimmune, and cardiovascular diseases. As a result, new technologies with the capability to selectively detect apoptotic cells represent a central focus of research for the study of these conditions. We have developed a new biosensor for the detection of apoptotic cells, incorporating the targeted selectivity for apoptotic cells from Annexin V with the sensitivity of bioluminescence signal generation from a serum-stable mutant of Renilla luciferase (RLuc8)...
May 25, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28541963/correlation-of-structural-and-functional-outcome-measures-in-a-phase-one-trial-of-ciliary-neurotrophic-factor-in-type-2-idiopathic-macular-telangiectasia
#15
Ferenc B Sallo, Irene Leung, Traci E Clemons, Tunde Peto, Emily Y Chew, Daniel Pauleikhoff, Alan C Bird
PURPOSE: Macular telangiectasia Type 2 is a bilateral, progressive, potentially blinding retinal disease characterized by both vascular and neurodegenerative signs. Both the area of the break in the ellipsoid zone seen in "en face" optical coherence tomographic (OCT) images and microperimetric focal retinal sensitivity loss have been proposed as potential measures of progression in macular telangiectasia. The authors aimed to assess the characteristics and interrelationship of these structural and functional disease markers from the data collected in a phase one clinical trial of ciliary neurotrophic factor in macular telangiectasia...
May 23, 2017: Retina
https://www.readbyqxmd.com/read/28541959/longitudinal-correlation-of-ellipsoid-zone-loss-and-functional-loss-in-macular-telangiectasia-type-2
#16
Tjebo F C Heeren, Diána Kitka, Daniela Florea, Traci E Clemons, Emily Y Chew, Alan C Bird, Daniel Pauleikhoff, Peter Charbel Issa, Frank G Holz, Tunde Peto
PURPOSE: To compare ellipsoid zone (EZ) loss and functional loss in macular telangiectasia (MacTel) type 2 longitudinally. METHODS: Prospective natural history study. Ellipsoid zone loss was measured in en-face images created from spectral domain optical coherence tomography. Functional loss was assessed by best-corrected visual acuity and microperimetry, counting the number of test points with impaired function. RESULTS: A total of 56 eyes of 31 participants were followed for 4...
May 24, 2017: Retina
https://www.readbyqxmd.com/read/28541476/7-8-dihydroxyflavone-ameliorates-cognitive-and-motor-deficits-in-a-huntington-s-disease-mouse-model-through-specific-activation-of-the-plc%C3%AE-1-pathway
#17
Gerardo García-Díaz Barriga, Albert Giralt, Marta Anglada-Huguet, Nuria Gaja-Capdevila, Javier G Orlandi, Jordi Soriano, Josep-Maria Canals, Jordi Alberch
Huntington's disease (HD) is a fatal neurodegenerative disease with motor, cognitive and psychiatric impairment. Dysfunctions in HD models have been related to reduced levels of striatal brain-derived neurotrophic factor (BDNF) and imbalance between its receptors TrkB and p75(NTR). Thus, molecules with activity on the BDNF/TrkB/p75 system can have therapeutic potential. 7,8-Dihydroxyflavone (7,8-DHF) was described as a TrkB agonist in several models of neuro-degenerative diseases, however, its TrkB activation profile needs further investigation due to its pleiotropic properties and divergence from BDNF effect...
May 24, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28541025/structural-genomic-variations-and-parkinson-s-disease
#18
Sara Bandrés-Ciga, Clara Ruz, Francisco J Barrero, Francisco Escamilla-Sevilla, Javier Pelegrina, Francisco Vives, Raquel Duran
Parkinson's disease (PD) is the second most common neurodegenerative disease, whose prevalence is projected to be between 8.7 and 9.3 million by 2030. Until about 20 years ago, PD was considered to be the textbook example of a "nongenetic" disorder. Nowadays, PD is generally considered a multifactorial disorder that arises from the combination and complex interaction of genes and environmental factors. To date, a total of 7 genes including SNCA, LRRK2, PARK2, DJ-1, PINK 1, VPS35 and ATP13A2 have been seen to cause unequivocally Mendelian PD...
May 25, 2017: Minerva Medica
https://www.readbyqxmd.com/read/28540926/entorhinal-cortical-deep-brain-stimulation-rescues-memory-deficits-in-both-young-and-old-mice-genetically-engineered-to-model-alzheimer-s-disease
#19
Frances Xia, Adelaide Yiu, Scellig Sd Stone, Soojin Oh, Andres M Lozano, Sheena A Josselyn, Paul W Frankland
Alzheimer's disease (AD) is a neurodegenerative disorder characterized by progressive cognitive decline. Deep brain stimulation (DBS) has been used to treat a variety of brain disorders and shows promise in alleviating cognitive symptoms in some AD patients (Laxton et al., 2010). We previously showed that DBS of the entorhinal cortex (EC) enhances spatial memory formation in normal (wild-type) mice (Stone et al., 2011). Here we tested the effects of EC-DBS on the progressive cognitive deficits in a genetically-based mouse model of AD...
May 25, 2017: Neuropsychopharmacology: Official Publication of the American College of Neuropsychopharmacology
https://www.readbyqxmd.com/read/28540772/management-of-glaucoma-as-a-neurodegenerative-disease
#20
Gurjeet Jutley, Sheila Mh Luk, Mohammad H Dehabadi, M Francesca Cordeiro
Glaucoma is a neurodegenerative disease with an estimated prevalence of 60 million people, and the most common cause of irreversible blindness worldwide. The mainstay of treatment has been aimed at lowering intraocular pressure, currently the only modifiable risk factor. Unfortunately, despite adequate pressure control, many patients go on to suffer irreversible visual loss. We first briefly examine currently established intraocular pressure lowering-treatments, with a discussion of their roles in neuroprotection as demonstrated by both animal and clinical studies...
April 2017: Neurodegenerative Disease Management
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