Cerebrospinal fluid beta-glucocerebrosidase activity is reduced in Dementia with Lewy Bodies.

abstract：:A novel oxime platform, the substituted phenoxyalkyl pyridinium oximes (US patent 9,227,937), was invented at Mississippi State University with an objective of discovering a brain-penetrating antidote to highly potent organophosphate anticholinesterases, such as the nerve agents. The goal was reactivation of inhibited...

journal_title：Neurobiology of disease

authors： Chambers JE,Meek EC

更新日期：2020-01-01 00:00:00

abstract：:Precursor cells in the adult dentate gyrus are a heterogeneous population. Astrocytic cell types with radial glia-like morphology and low proliferative activity have been distinguished from highly dividing subtypes expressing early neuronal properties. Recent evidence indicates that physiological stimuli predominantly...

journal_title：Neurobiology of disease

authors： Kunze A,Grass S,Witte OW,Yamaguchi M,Kempermann G,Redecker C

更新日期：2006-02-01 00:00:00

abstract：:TDP-43 proteinopathy is very prevalent among the elderly (affecting at least 25% of individuals over 85 years of age) and is associated with substantial cognitive impairment. Risk factors implicated in age-related TDP-43 proteinopathy include commonly inherited gene variants, comorbid Alzheimer's disease pathology, an...

journal_title：Neurobiology of disease

authors： Nelson PT,Gal Z,Wang WX,Niedowicz DM,Artiushin SC,Wycoff S,Wei A,Jicha GA,Fardo DW

更新日期：2019-05-01 00:00:00

abstract：:Aging is the strongest risk factor for metabolic, vascular and neurodegenerative diseases. Aging alone is associated with a gradual decline of cognitive and motor functions. Considering an increasing elderly population in the last century, understanding the cellular and molecular mechanisms contributing to brain aging...

journal_title：Neurobiology of disease

authors： Salas IH,Burgado J,Allen NJ

更新日期：2020-09-01 00:00:00

abstract：:The pathophysiology of Parkinson's disease (PD) and of L-DOPA-induced dyskinesia (LID) is associated with dysfunctional neuronal activity in several nuclei of the basal ganglia. Moreover, high levels of oscillatory activity and synchronization have also been described in both intra- and inter-basal ganglia nuclei and ...

journal_title：Neurobiology of disease

authors： Aristieta A,Ruiz-Ortega JA,Miguelez C,Morera-Herreras T,Ugedo L

更新日期：2016-05-01 00:00:00

abstract：:The choroid plexus contains a major reservoir of macrophages poised for efficient delivery of virus and neurotoxins to the brain after infection by lentiviruses such as human or feline immunodeficiency virus (FIV). However, their contribution to neurotoxicity is poorly understood. Medium from FIV-infected, choroid ple...

journal_title：Neurobiology of disease

authors： Bragg DC,Boles JC,Meeker RB

更新日期：2002-03-01 00:00:00

abstract：:Alzheimer's disease (AD) is characterized by overproduction of A beta derived from APP cleavage via beta- and gamma-secretase pathway. Recent evidence has linked altered cholesterol metabolism to AD pathogenesis. In this study, we show that AD brain had significant cholesterol retention and high beta- and gamma-secret...

journal_title：Neurobiology of disease

authors： Xiong H,Callaghan D,Jones A,Walker DG,Lue LF,Beach TG,Sue LI,Woulfe J,Xu H,Stanimirovic DB,Zhang W

更新日期：2008-03-01 00:00:00

abstract：:Recently, we showed that oxidative stress activates the expression and activity of the beta-site AbetaPP-cleaving enzyme (BACE), an aspartyl protease responsible for the beta-secretase cleavage of AbetaPP. The identification of compounds able to prevent the induction of this event is an important goal of therapeutic s...

journal_title：Neurobiology of disease

authors： Tamagno E,Guglielmotto M,Bardini P,Santoro G,Davit A,Di Simone D,Danni O,Tabaton M

更新日期：2003-11-01 00:00:00

abstract：:Charcot-Marie-Tooth disease is a commonly inherited form of neuropathy. Although named over 100 years ago, identification of subtypes of Charcot-Marie-Tooth has rapidly expanded in the preceding decades with the advancement of genetic sequencing, including type 2F (CMT2F), due to mutations in heat shock protein 27 (Hs...

journal_title：Neurobiology of disease

authors： Schwartz NU

更新日期：2019-10-01 00:00:00

abstract：:Recent large-scale genetic studies have allowed for the first glimpse of the effects of common genetic variability in dementia with Lewy bodies (DLB), identifying risk variants with appreciable effect sizes. However, it is currently well established that a substantial portion of the genetic heritable component of comp...

journal_title：Neurobiology of disease

authors： Guerreiro R,Escott-Price V,Hernandez DG,Kun-Rodrigues C,Ross OA,Orme T,Neto JL,Carmona S,Dehghani N,Eicher JD,Shepherd C,Parkkinen L,Darwent L,Heckman MG,Scholz SW,Troncoso JC,Pletnikova O,Dawson T,Rosenthal L,Ansor

更新日期：2019-07-01 00:00:00

abstract：:Based on in vitro studies, it is hypothesized that neurotrophic factor deprivation following deafferentation elicits an oxidative state change in the deafferented neuron and the formation of free radicals that then signal cell death pathways. This pathway to cell death was tested in vivo by assessing the efficacy of a...

journal_title：Neurobiology of disease

authors： Maruyama J,Yamagata T,Ulfendahl M,Bredberg G,Altschuler RA,Miller JM

更新日期：2007-02-01 00:00:00

abstract：:Somatosensation plays a critical role in the dexterous manipulation of objects, in emotional communication, and in the embodiment of our limbs. For upper-limb neuroprostheses to be adopted by prospective users, prosthetic limbs will thus need to provide sensory information about the position of the limb in space and a...

journal_title：Neurobiology of disease

authors： Tabot GA,Kim SS,Winberry JE,Bensmaia SJ

更新日期：2015-11-01 00:00:00

abstract：:Glutamate excitotoxicity is a consolidated hypothesis in neonatal brain injuries and tissue plasminogen activator (t-PA) participates in the processes through proteolytic and receptor mediated effects. In brain microvascular endothelial cell (nBMEC) cultures from neonates, t-PA content and release upon glutamate are h...

journal_title：Neurobiology of disease

authors： Henry VJ,Lecointre M,Laudenbach V,Ali C,Macrez R,Jullienne A,Berezowski V,Carmeliet P,Vivien D,Marret S,Gonzalez BJ,Leroux P

更新日期：2013-02-01 00:00:00

abstract：:Mechanisms determining characteristic age-of-onset for neurological diseases are largely unknown. Normal brain aging associates with robust and progressive transcriptome changes ("molecular aging"), but the intersection with disease pathways is mostly uncharacterized. Here, using cross-cohort microarray analysis of fo...

journal_title：Neurobiology of disease

authors： Glorioso C,Oh S,Douillard GG,Sibille E

更新日期：2011-02-01 00:00:00

abstract：:Amyotrophic lateral sclerosis (ALS) is characterised by substantial loss of both upper and lower motor neuron function, with sensory and cognitive systems less affected. Though heritable forms of the disease have been described, the vast majority of cases are sporadic with poorly defined underlying pathogenic mechanis...

journal_title：Neurobiology of disease

authors： Peters OM,Millership S,Shelkovnikova TA,Soto I,Keeling L,Hann A,Marsh-Armstrong N,Buchman VL,Ninkina N

更新日期：2012-10-01 00:00:00

abstract：:During the last few years, adenoviral gene transfer techniques have achieved increasing interest in the treatment of neurodegenerative diseases. However, gene therapy requires that delivered genes are translated into proteins. This may pose a problem in focal ischemia where protein synthesis is compromized. The presen...

journal_title：Neurobiology of disease

authors： Hermann DM,Kilic E,Kügler S,Isenmann S,Bähr M

更新日期：2001-08-01 00:00:00

abstract：:Amyloid beta-peptide (Abeta) containing plaques and neurofibrillary tangles (NFT) are the two major histopathological hallmarks of Alzheimer's disease (AD). According to the amyloid cascade hypothesis, deposition of Abeta is an initial and essential step in the pathogenesis of AD, and formation of NFT has been propose...

journal_title：Neurobiology of disease

authors： Kulic L,Kurosinski P,Chen F,Tracy J,Mohajeri MH,Li H,Nitsch RM,Götz J

更新日期：2006-04-01 00:00:00

abstract：:Insulin deficiency may contribute toward the neurological deficits of diabetic polyneuropathy (DPN). In particular, the unique trophic properties of insulin, acting on sensory neuron and axon receptors offer an approach toward reversing loss of skin axons that develops during diabetes. Here we examined how local cutan...

journal_title：Neurobiology of disease

authors： Guo G,Kan M,Martinez JA,Zochodne DW

更新日期：2011-08-01 00:00:00

abstract：:To explore mechanisms of epileptogenesis in audiogenic seizures (AGS), we examined the expression of alpha-amino-3-hydroxy-5-methyl-4-isoxazoleopropionic acid (AMPA) receptor subunits GluR1 and GluR2 and of the GluR-associated protein Narp in the hippocampus and the inferior colliculus (IC) from AGS-susceptible P77PMC...

journal_title：Neurobiology of disease

authors： Li SY,Xu DS,Jia HT

更新日期：2003-12-01 00:00:00

abstract：:In this study, we have shown that a paraneoplastic cerebellar degeneration (PCD)-associated antigen, pcd17, binds to a cell cycle-related protein, MRG15. MRG15 derepresses the E2F-responsive B-myb promoter. The pcd17 antigen inhibits the derepression of the B-myb transcriptional activity by MRG15, and, as a result, pc...

journal_title：Neurobiology of disease

authors： Sakai K,Kitagawa Y,Saiki S,Saiki M,Hirose G

更新日期：2004-04-01 00:00:00

abstract：:Pathological gambling (PG) represents a behavioral side effect of dopamine replacement therapy in a minority of patients with Parkinson's disease (PD). Using striatal dopamine transporter (DAT) with single photon emission tomography we assessed presynaptic dopaminergic function in 8 PD patients with PG, 21 matched PD ...

journal_title：Neurobiology of disease

authors： Cilia R,Ko JH,Cho SS,van Eimeren T,Marotta G,Pellecchia G,Pezzoli G,Antonini A,Strafella AP

更新日期：2010-07-01 00:00:00

abstract：:Genetic and biochemical abnormalities associated with alpha-synuclein are implicated in the etiology of Parkinson's disease (PD). In this study, altered locomotor behavior linked to the expression of mutant or wildtype human alpha-synuclein was investigated. A53T alpha-synuclein transgenic (A53T-tg) mice exhibited nor...

journal_title：Neurobiology of disease

authors： Unger EL,Eve DJ,Perez XA,Reichenbach DK,Xu Y,Lee MK,Andrews AM

更新日期：2006-02-01 00:00:00

abstract：:Mutations in the gene encoding the RNA-binding protein TDP-43 cause amyotrophic lateral sclerosis (ALS), clinically and pathologically indistinguishable from the majority of 'sporadic' cases of ALS, establishing altered TDP-43 function and distribution as a primary mechanism of neurodegeneration. Transgenic mouse mode...

journal_title：Neurobiology of disease

authors： Gordon D,Dafinca R,Scaber J,Alegre-Abarrategui J,Farrimond L,Scott C,Biggs D,Kent L,Oliver PL,Davies B,Ansorge O,Wade-Martins R,Talbot K

更新日期：2019-01-01 00:00:00

abstract：:Recently an aspartyl protease with beta-secretase activity called BACE was identified. In the present paper we showed that BACE is modulated by the oxidative stress product 4-hydroxynonenal (HNE). Exposure of NT(2) neurons to the two classical pro-oxidant stimuli ascorbate/FeSO(4) and H(2)O(2)/FeSO(4) resulted in a si...

journal_title：Neurobiology of disease

authors： Tamagno E,Bardini P,Obbili A,Vitali A,Borghi R,Zaccheo D,Pronzato MA,Danni O,Smith MA,Perry G,Tabaton M

更新日期：2002-08-01 00:00:00

abstract：:Exaggerated activity in the beta band (13-35 Hz) is a hallmark of basal ganglia signals in patients with Parkinson's disease (PD). Beta activity however is not constantly elevated, but comes in bursts. In previous work we showed that the longer beta bursts are maintained, the more the oscillatory synchronisation withi...

journal_title：Neurobiology of disease

authors： Tinkhauser G,Torrecillos F,Duclos Y,Tan H,Pogosyan A,Fischer P,Carron R,Welter ML,Karachi C,Vandenberghe W,Nuttin B,Witjas T,Régis J,Azulay JP,Eusebio A,Brown P

更新日期：2018-09-01 00:00:00

abstract：:While much of the research on neurodegenerative diseases has focused on neurons, non-neuronal cells are also affected. The extent to which glia and other non-neuronal cells are causally involved in disease pathogenesis versus more passively responding to disease is an area of active research. This is complicated by th...

journal_title：Neurobiology of disease

authors： Gleichman AJ,Carmichael ST

更新日期：2020-08-01 00:00:00

abstract：:Giliosis is a hallmark of prion disease. A neurotoxic prion peptide (PrP106-126) induces astrocyte proliferation in the presence of microglia. This peptide also directly enhances microglial proliferation in culture. We have investigated this further to understand the method by which factors released by microglia and P...

journal_title：Neurobiology of disease

authors： Brown DR,Schmidt B,Kretzschmar HA

更新日期：1998-04-01 00:00:00

abstract：:Huntington's disease (HD) is a lethal, neurodegenerative disorder caused by expansion of the polyglutamine repeat in the Huntingtin gene (HTT), leading to mutant protein misfolding, aggregation, and neuronal death. Feeding a Drosophila HD model cystamine, or expressing a transgene encoding the anti-htt intracellular a...

journal_title：Neurobiology of disease

authors： Bortvedt SF,McLear JA,Messer A,Ahern-Rindell AJ,Wolfgang WJ

更新日期：2010-10-01 00:00:00

abstract：:CLN5 disease, late infantile variant phenotype neuronal ceroid lipofuscinosis, is a severe neurodegenerative disease caused by mutations in the CLN5 gene, which encodes a lysosomal protein of unknown function. Cln5-deficiency in mice leads to loss of thalamocortical neurons, and glial activation, but the underlying me...

journal_title：Neurobiology of disease

authors： Schmiedt ML,Blom T,Blom T,Kopra O,Wong A,von Schantz-Fant C,Ikonen E,Kuronen M,Jauhiainen M,Cooper JD,Jalanko A

更新日期：2012-04-01 00:00:00

abstract：:Following intracerebral inoculation, the BeAn 8386 strain of Theiler's virus causes persistent infection and inflammatory demyelinating encephalomyelitis in the spinal cord of T-cell defective SJL/J mice, which is widely used as a model of multiple sclerosis. In contrast, C57BL/6 (B6) mice clear the virus and develop ...

journal_title：Neurobiology of disease

authors： Bröer S,Hage E,Käufer C,Gerhauser I,Anjum M,Li L,Baumgärtner W,Schulz TF,Löscher W

更新日期：2017-03-01 00:00:00