Remodeling of striatal NMDA receptors by chronic A(2A) receptor blockade in Huntington's disease mice.

abstract：:The neuroprotective effect of L-kynurenine sulfate (KYN), a precursor of kynurenic acid (KYNA, a selective N-methyl-D-aspartate receptor antagonist), was studied. KYN (300 mg/kg i.p., applied daily for 5 days) appreciably decreased the number of injured pyramidal cells from 1850+/-100/mm(2) to 1000+/-300/mm(2) (p<0.00...

journal_title：Neurobiology of disease

authors： Sas K,Robotka H,Rózsa E,Agoston M,Szénási G,Gigler G,Marosi M,Kis Z,Farkas T,Vécsei L,Toldi J

更新日期：2008-11-01 00:00:00

abstract：:Down syndrome (DS) is the most common genetic disorder associated with mental retardation. It has been repeatedly shown that Ts65Dn mice, the major animal model for DS, have severe cognitive and synaptic plasticity dysfunctions caused by excessive inhibition in their temporal lobe structures. Here we employed a multid...

journal_title：Neurobiology of disease

authors： Begenisic T,Baroncelli L,Sansevero G,Milanese M,Bonifacino T,Bonanno G,Cioni G,Maffei L,Sale A

更新日期：2014-03-01 00:00:00

abstract：:In this review, we describe insights into beta-amyloid (Abeta) production using aged dogs as a model of human brain aging. The advantage of using dogs is that they naturally accumulate Abeta neuropathology with age. In parallel, dogs also develop age-associated learning and memory impairments. Thus, dogs can complemen...

journal_title：Neurobiology of disease

authors： Head E,Torp R

更新日期：2002-02-01 00:00:00

abstract：:Human immunodeficiency virus (HIV) infection continues to rise in drug-abusing populations and causes a dementing illness in a subset of individuals. Factors contributing to the development of dementia in this population remain unknown. We found that HIV-infected individuals with the E4 allele of Apolipoprotein E (Apo...

journal_title：Neurobiology of disease

authors： Turchan-Cholewo J,Liu Y,Gartner S,Reid R,Jie C,Peng X,Chen KC,Chauhan A,Haughey N,Cutler R,Mattson MP,Pardo C,Conant K,Sacktor N,McArthur JC,Hauser KF,Gairola C,Nath A

更新日期：2006-07-01 00:00:00

abstract：:Butyrylcholinesterase (BChE) genotypes and protein (BuChE) activity, especially in combination with Apolipoprotein E4 (ApoE4), have been investigated as risk factors for developing Alzheimer disease (AD) and may be associated with the rate of progression of cognitive decline. Despite similar pathologic (e.g. amyloid d...

journal_title：Neurobiology of disease

authors： Maetzler W,Keller S,Michelis J,Koehler N,Stransky E,Becker C,Schulte C,Melms A,Gasser T,Berg D

更新日期：2009-08-01 00:00:00

abstract：:4-Aminopyridine (4AP, 50 μM) induces interictal- and ictal-like discharges in brain slices including parahippocampal areas such as the entorhinal cortex (EC) but the relation between these two types of epileptiform activity remains undifined. Here, by employing field potential recordings in rat EC slices during 4AP ap...

journal_title：Neurobiology of disease

authors： Avoli M,Panuccio G,Herrington R,D'Antuono M,de Guzman P,Lévesque M

更新日期：2013-04-01 00:00:00

abstract：:Chronic microglial activation has been linked to the progressive degeneration of the nigrostriatal dopaminergic neurons evidenced in Parkinson's disease (PD) pathogenesis. The exact etiology of PD remains poorly understood. Although both oxidative stress and neuroinflammation are identified as co-contributors in PD pa...

journal_title：Neurobiology of disease

authors： Gordon R,Singh N,Lawana V,Ghosh A,Harischandra DS,Jin H,Hogan C,Sarkar S,Rokad D,Panicker N,Anantharam V,Kanthasamy AG,Kanthasamy A

更新日期：2016-09-01 00:00:00

abstract：:Frontotemporal dementia (FTD) is typified by behavioral and cognitive changes manifested as altered social comportment and impaired memory performance. To investigate the neurodegenerative consequences of progranulin gene (GRN) mutations, which cause an inherited form of FTD, we used previously generated progranulin k...

journal_title：Neurobiology of disease

authors： Ghoshal N,Dearborn JT,Wozniak DF,Cairns NJ

更新日期：2012-01-01 00:00:00

abstract：:Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is a rare type of leukodystrophy caused by mutations in either MLC1 or GLIALCAM genes. Previous work indicated that chloride currents mediated by the volume-regulated anion channel (VRAC) and ClC-2 channels were affected in astrocytes deficient in either...

journal_title：Neurobiology of disease

authors： Elorza-Vidal X,Sirisi S,Gaitán-Peñas H,Pérez-Rius C,Alonso-Gardón M,Armand-Ugón M,Lanciotti A,Brignone MS,Prat E,Nunes V,Ambrosini E,Gasull X,Estévez R

更新日期：2018-11-01 00:00:00

abstract：:Parkinson's disease (PD) is a neurodegenerative disorder caused by loss of dopaminergic neurons. Although many reports have suggested that genetic factors are implicated in the pathogenesis of PD, molecular mechanisms underlying selective dopaminergic neuronal degeneration remain unknown. DJ-1 is a causative gene for ...

journal_title：Neurobiology of disease

authors： Usami Y,Hatano T,Imai S,Kubo S,Sato S,Saiki S,Fujioka Y,Ohba Y,Sato F,Funayama M,Eguchi H,Shiba K,Ariga H,Shen J,Hattori N

更新日期：2011-09-01 00:00:00

abstract：:Hereditary sensory neuropathy type 1 (HSN-1) is a peripheral neuropathy most frequently caused by mutations in the SPTLC1 or SPTLC2 genes, which code for two subunits of the enzyme serine palmitoyltransferase (SPT). SPT catalyzes the first step of de novo sphingolipid synthesis. Mutations in SPT result in a change in ...

journal_title：Neurobiology of disease

authors： Wilson ER,Kugathasan U,Abramov AY,Clark AJ,Bennett DLH,Reilly MM,Greensmith L,Kalmar B

更新日期：2018-09-01 00:00:00

abstract：:The central nervous system is protected by barriers which control the entry of compounds into the brain, thereby regulating brain homeostasis. The blood-brain barrier, formed by the endothelial cells of the brain capillaries, restricts access to brain cells of blood-borne compounds and facilitates nutrients essential ...

journal_title：Neurobiology of disease

authors： Gabathuler R

更新日期：2010-01-01 00:00:00

abstract：:Exposure to stress during pregnancy influences the trajectory of brain development resulting in permanent alterations that may contribute to increased susceptibility to subsequent cognitive or neuropsychiatric disorders. In this manuscript, we examined the effects of prenatal stress on the expression of basic fibrobla...

journal_title：Neurobiology of disease

authors： Fumagalli F,Bedogni F,Slotkin TA,Racagni G,Riva MA

更新日期：2005-12-01 00:00:00

abstract：:Infantile neuronal ceroid lipofuscinosis (INCL) is a severe neurodegenerative disorder of children, characterized by selective death of neocortical neurons. To understand early disease mechanisms in INCL, we have studied Ppt1(Deltaex4) knock-out mouse neurons in culture and acute brain slices. Global transcript profil...

journal_title：Neurobiology of disease

authors： Ahtiainen L,Kolikova J,Mutka AL,Luiro K,Gentile M,Ikonen E,Khiroug L,Jalanko A,Kopra O

更新日期：2007-10-01 00:00:00

abstract：:Infection of immunocompetent adult rats with Borna disease virus (BDV) causes severe encephalitis and neural dysfunction. The expression of COX-2 and CGRP, genes previously shown to be implicated in CNS disease and peripheral inflammation, was dramatically upregulated in the cortical neurons of acutely BDV-infected ra...

journal_title：Neurobiology of disease

authors： Röhrenbeck AM,Bette M,Hooper DC,Nyberg F,Eiden LE,Dietzschold B,Weihe E

更新日期：1999-02-01 00:00:00

abstract：:Ubiquitination, a fundamental post-translational modification of intracellular proteins, is enzymatically reversed by deubiquitinase enzymes (deubiquitinases). >90 deubiquitinases have been identified. One of these enzymes, YOD1, possesses deubiquitinase activity and is similar to ovarian tumor domain-containing prote...

journal_title：Neurobiology of disease

authors： Tanji K,Mori F,Miki Y,Utsumi J,Sasaki H,Kakita A,Takahashi H,Wakabayashi K

更新日期：2018-04-01 00:00:00

abstract：:The hippocampus is often injured in neonatal stroke. We have investigated the effect of erythropoietin (EPO) on oxygen-glucose deprived hippocampal slices and hypoxic progenitor cells. EPO improved survival of the organotypic hippocampal slices with significantly less cell death in the dentate gyrus and an increased n...

journal_title：Neurobiology of disease

authors： Osredkar D,Sall JW,Bickler PE,Ferriero DM

更新日期：2010-05-01 00:00:00

abstract：:The focus on amyloid plaques and neurofibrillary tangles has yielded no Alzheimer's disease (AD) modifying treatments in the past several decades, despite successful studies in preclinical mouse models. This inconsistency has caused a renewed focus on improving the fidelity and reliability of AD mouse models, with dis...

journal_title：Neurobiology of disease

authors： Lewandowski CT,Maldonado Weng J,LaDu MJ

更新日期：2020-06-01 00:00:00

abstract：:Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (PLOSL) is a recessively inherited disease characterized by early onset dementia associated with bone cysts. Our group has recently established the molecular background of PLOSL by identifying mutations in DAP12 and TREM2 genes. To understand...

journal_title：Neurobiology of disease

authors： Kiialainen A,Hovanes K,Paloneva J,Kopra O,Peltonen L

更新日期：2005-03-01 00:00:00

abstract：:Multiple sclerosis (MS) is the prototypical inflammatory disease of the central nervous system (CNS), leading to multifocal demyelination and neurodegeneration. The etiology of this incurable disease is unknown and remains a matter of intensive research. The possibility that microbial infections, such as viruses or ba...

journal_title：Neurobiology of disease

authors： Alonso R,Fernández-Fernández AM,Pisa D,Carrasco L

更新日期：2018-09-01 00:00:00

abstract：:Mutations in the voltage-gated sodium channel SCN1A are responsible for a number of seizure disorders including Generalized Epilepsy with Febrile Seizures Plus (GEFS+) and Severe Myoclonic Epilepsy of Infancy (SMEI). To determine the effects of SCN1A mutations on channel function in vivo, we generated a bacterial arti...

journal_title：Neurobiology of disease

authors： Tang B,Dutt K,Papale L,Rusconi R,Shankar A,Hunter J,Tufik S,Yu FH,Catterall WA,Mantegazza M,Goldin AL,Escayg A

更新日期：2009-07-01 00:00:00

abstract：:No single-omic approach completely elucidates the multitude of alterations taking place in Alzheimer's disease (AD). Here, we coupled transcriptomic and phosphoproteomic approaches to determine the temporal sequence of changes in mRNA, protein, and phosphopeptide expression levels from human temporal cortical samples,...

journal_title：Neurobiology of disease

authors： Marttinen M,Paananen J,Neme A,Mitra V,Takalo M,Natunen T,Paldanius KMA,Mäkinen P,Bremang M,Kurki MI,Rauramaa T,Leinonen V,Soininen H,Haapasalo A,Pike I,Hiltunen M

更新日期：2019-04-01 00:00:00

abstract：:Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by motor neuron death. A 20% of familial ALS cases are associated with mutations in the gene coding for superoxide dismutase 1 (SOD1). The accumulation of abnormal aggregates of different proteins is a common feature in motor neurons of p...

journal_title：Neurobiology of disease

authors： Pinto C,Medinas DB,Fuentes-Villalobos F,Maripillán J,Castro AF,Martínez AD,Osses N,Hetz C,Henríquez JP

更新日期：2019-10-01 00:00:00

abstract：:Amyotrophic lateral sclerosis (ALS) is a chronic neurodegenerative disease characterized by the progressive loss of motor neurons, leading to profound weakness and eventual death of affected individuals. For the vast majority of patients with ALS, the etiology of the disorder is unknown, and although multiple clinical...

journal_title：Neurobiology of disease

authors： Elliott JL

更新日期：1999-10-01 00:00:00

abstract：:The most appropriate time for the initiation of dopaminergic symptomatic therapy in Parkinson's disease remains debatable. It has been suggested that early correction of basal ganglia pathophysiological abnormalities may have long-term beneficial effects. To test this hypothesis, we investigated the early and delayed ...

journal_title：Neurobiology of disease

authors： Marin C,Bonastre M,Mengod G,Cortés R,Giralt A,Obeso JA,Schapira AH

更新日期：2014-04-01 00:00:00

abstract：:The neuropathology of hippocampal seizure foci in human temporal lobe epilepsy (TLE) and several animal models of epilepsy reveal extensive neuronal loss along with astrocyte and microglial activation. Studies of these models have advanced hypotheses that propose both pathological changes are essential for seizure gen...

journal_title：Neurobiology of disease

authors： Lee TS,Li AY,Rapuano A,Mantis J,Eid T,Seyfried TN,de Lanerolle NC

更新日期：2021-01-01 00:00:00

abstract：:β-Amyloid (Aβ) plaques in Alzheimer (AD) brains are surrounded by severe dendritic and axonal changes, including local spine loss, axonal swellings and distorted neurite trajectories. Whether and how plaques induce these neuropil abnormalities remains unknown. We tested the hypothesis that oligomeric assemblies of Aβ,...

journal_title：Neurobiology of disease

authors： DaRocha-Souto B,Coma M,Pérez-Nievas BG,Scotton TC,Siao M,Sánchez-Ferrer P,Hashimoto T,Fan Z,Hudry E,Barroeta I,Serenó L,Rodríguez M,Sánchez MB,Hyman BT,Gómez-Isla T

更新日期：2012-01-01 00:00:00

abstract：:Fragile X syndrome (FXS) patients do not make the fragile X mental retardation protein (FMRP). The absence of FMRP causes dysregulated translation, abnormal synaptic plasticity and the most common form of inherited intellectual disability. But FMRP loss has minimal effects on memory itself, making it difficult to unde...

journal_title：Neurobiology of disease

authors： Radwan B,Dvorak D,Fenton AA

更新日期：2016-04-01 00:00:00

abstract：:To thoroughly understand the function and regulation of neurotransmitter systems in the brain, as well as the underlying disease mechanisms, it is important to comprehensively analyze the expression patterns of genes participating in such systems. Using functional annotated cDNA clones (FANTOM), we examined the gene e...

journal_title：Neurobiology of disease

authors： Ichikawa M,Okamura-Oho Y,Okunishi R,Kanamori M,Suzuki H,Ritani A,Nitta H,Eguchi N,Urade Y,Hayashizaki Y

更新日期：2005-08-01 00:00:00

abstract：:Huntington disease (HD) is an adult onset, neurodegenerative disorder that results from CAG expansion in the HD gene. Recent work has demonstrated testicular degeneration in mouse models of HD and alterations in the hypothalamic-pituitary-gonadal (HPG) axis in HD patients. Here, we show that HD patients have specific ...

journal_title：Neurobiology of disease

authors： Van Raamsdonk JM,Murphy Z,Selva DM,Hamidizadeh R,Pearson J,Petersén A,Björkqvist M,Muir C,Mackenzie IR,Hammond GL,Vogl AW,Hayden MR,Leavitt BR

更新日期：2007-06-01 00:00:00