AGS-induced expression of Narp is concomitant with expression of AMPA receptor subunits GluR1 and GluR2 in hippocampus but not inferior colliculus of P77PMC rats.

abstract：OBJECTIVE:To investigate structural connectivity and the relationship between axonal microstructure and clinical, cognitive, and motor functions in premanifest (pre-HD) and symptomatic (symp-HD) Huntington's disease. METHOD:Diffusion tensor imaging (DTI) data were acquired from 35 pre-HD, 36 symp-HD, and 35 controls. ...

journal_title：Neurobiology of disease

authors： Poudel GR,Stout JC,Domínguez D JF,Salmon L,Churchyard A,Chua P,Georgiou-Karistianis N,Egan GF

更新日期：2014-05-01 00:00:00

abstract：:Parkinson's Disease (PD) and Multiple System Atrophy (MSA) are neurodegenerative diseases characterized neuropathologically by alpha-synuclein accumulation in brain cells. This accumulation is hypothesized to contribute to constitutive neuroinflammation, and to participate in the neurodegeneration. Cytokines, which ar...

journal_title：Neurobiology of disease

authors： Rydbirk R,Elfving B,Andersen MD,Langbøl MA,Folke J,Winge K,Pakkenberg B,Brudek T,Aznar S

更新日期：2017-10-01 00:00:00

abstract：:The autophagy-lysosomal degradation pathway plays a role in the onset and progression of neurodegenerative diseases. Clinical and genetic studies indicate that mutations of beta-glucocerebrosidase represent genetic risk factors for synucleinopathies, including Parkinson's Disease (PD) and Dementia with Lewy Bodies (DL...

journal_title：Neurobiology of disease

authors： Parnetti L,Balducci C,Pierguidi L,De Carlo C,Peducci M,D'Amore C,Padiglioni C,Mastrocola S,Persichetti E,Paciotti S,Bellomo G,Tambasco N,Rossi A,Beccari T,Calabresi P

更新日期：2009-06-01 00:00:00

abstract：:Intermittent hypoxia (IH) during sleep induces temporally defined increases in apoptosis within vulnerable brain regions such as the hippocampal CA1 region in rats. Protein kinase B (AKT) has emerged as major signal transduction protein underlying inhibition of apoptosis and consequent increases in cell survival. Spra...

journal_title：Neurobiology of disease

authors： Goldbart A,Cheng ZJ,Brittian KR,Gozal D

更新日期：2003-12-01 00:00:00

abstract：:Whether seizures in the developing brain cause long-term changes in the mature brain has been debated. We tested the hypothesis that a model of early-life seizures, induced by systemic injection of a GABA(B) receptor antagonist CGP56999A in immature rats, decreased GABA(B) receptor-mediated inhibitory postsynaptic cur...

journal_title：Neurobiology of disease

authors： Qu L,Boyce R,Leung LS

更新日期：2010-03-01 00:00:00

abstract：:The present study aimed to evaluate the therapeutic potential of clioquinol (CQ), a metal chelator, on multiple sclerosis pathogenesis. Experimental autoimmune encephalomyelitis was induced by immunization with myelin oligodendrocyte glycoprotein (MOG(35-55)) in female mice. Three weeks after the initial immunization,...

journal_title：Neurobiology of disease

authors： Choi BY,Jang BG,Kim JH,Seo JN,Wu G,Sohn M,Chung TN,Suh SW

更新日期：2013-06-01 00:00:00

abstract：:Experiments reported here were motivated by studies in both human epilepsy and animal models in which stunted dendritic arbors are observed. Our goal was to determine if chronic network hyperexcitability alters dendritic growth. Experiments were conducted in hippocampal slice cultures obtained from infant mice that ex...

journal_title：Neurobiology of disease

authors： Nishimura M,Owens J,Swann JW

更新日期：2008-02-01 00:00:00

abstract：:Nicotinamide adenine dinucleotide phosphate oxidase (NOX) is widely expressed in brain tissue including neurons, glia, and endothelia in neurovascular units. It is a major source of oxidants in the post-ischemic brain and significantly contributes to ischemic brain damage. Inflammation occurs after brain ischemia and ...

journal_title：Neurobiology of disease

authors： Chen H,Kim GS,Okami N,Narasimhan P,Chan PH

更新日期：2011-06-01 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：: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：:Duplication 15q syndrome (Dup15q) is an autism-associated disorder co-incident with high rates of pediatric epilepsy. Additional copies of the E3 ubiquitin ligase UBE3A are thought to cause Dup15q phenotypes, yet models overexpressing UBE3A in neurons have not recapitulated the epilepsy phenotype. We show that Drosoph...

journal_title：Neurobiology of disease

authors： Hope KA,LeDoux MS,Reiter LT

更新日期：2017-12-01 00:00:00

abstract：:Schizophrenia is a devastating neurodevelopmental disorder that affects approximately 1% of the population. Reduced expression of the 67-kDa protein isoform of glutamic acid decarboxylase (GAD67) is a hallmark of the disease and is encoded by the GAD1 gene. In schizophrenia, GAD67 downregulation occurs in multiple int...

journal_title：Neurobiology of disease

authors： Brown JA,Horváth S,Garbett KA,Schmidt MJ,Everheart M,Gellért L,Ebert P,Mirnics K

更新日期：2014-03-01 00:00:00

abstract：:Injury to retinal ganglion cell (RGC) axons triggers rapid activation of Jun N-terminal kinase (JNK) signaling, a major prodeath pathway in injured RGCs. Of the multiple kinases that can activate JNK, dual leucine kinase (Dlk) is known to regulate both apoptosis and Wallerian degeneration triggered by axonal insult. H...

journal_title：Neurobiology of disease

authors： Fernandes KA,Harder JM,John SW,Shrager P,Libby RT

更新日期：2014-09-01 00:00:00

abstract：:Mutations in the gene for DJ-1 have been associated with early-onset autosomal recessive parkinsonism. Previous studies of null DJ-1 mice have shown alterations in striatal dopamine (DA) transmission with no DAergic cell loss. Here we characterize a new line of DJ-1-deficient mice. A subtle locomotor deficit was prese...

journal_title：Neurobiology of disease

authors： Manning-Boğ AB,Caudle WM,Perez XA,Reaney SH,Paletzki R,Isla MZ,Chou VP,McCormack AL,Miller GW,Langston JW,Gerfen CR,Dimonte DA

更新日期：2007-08-01 00:00:00

abstract：:Mutations in APP (amyloid precursor protein), PSEN1 (presenilin 1) or PSEN2 (presenilin 2) are the main cause of early-onset familial forms of Alzheimer's disease (autosomal dominant AD or ADAD). These genes affect γ-secretase-dependent generation of Amyloid β (Aβ) peptides, the main constituent of amyloid plaques and...

journal_title：Neurobiology of disease

authors： Pimenova AA,Goate AM

更新日期：2020-05-01 00:00:00

abstract：:alpha-Synuclein has been identified as a major component of Lewy body inclusions, which are one of the pathologic hallmarks of idiopathic Parkinson's disease. Mutations in alpha-synuclein have been found to be responsible for rare familial cases of Parkinsonism. To test whether overexpression of human alpha-synuclein ...

journal_title：Neurobiology of disease

authors： Matsuoka Y,Vila M,Lincoln S,McCormack A,Picciano M,LaFrancois J,Yu X,Dickson D,Langston WJ,McGowan E,Farrer M,Hardy J,Duff K,Przedborski S,Di Monte DA

更新日期：2001-06-01 00:00:00

abstract：:The TATA-box binding protein associated factor 1 (TAF1) is part of the TFIID complex that plays a key role during the initiation of transcription. Variants of TAF1 are associated with neurodevelopmental disorders. Previously, we found that CRISPR/Cas9 based editing of the TAF1 gene disrupts the morphology of the cereb...

journal_title：Neurobiology of disease

authors： Dhanalakshmi C,Janakiraman U,Moutal A,Fukunaga K,Khanna R,Nelson MA

更新日期：2021-02-01 00:00:00

abstract：:It has been proposed that Alzheimer disease (AD) is associated with a "disconnection syndrome" due to the gradual loss of morphological and functional integrity of cortico-cortical pathways. This hypothesis derives from indirect neuropathological observations, but definitive evidence that AD primarily targets cortico-...

journal_title：Neurobiology of disease

authors： Delatour B,Blanchard V,Pradier L,Duyckaerts C

更新日期：2004-06-01 00:00:00

abstract：:G-protein coupled receptor 3 (GPR3), GPR6, and GPR12 belong to a family of constitutively active Gs-coupled receptors that activate 3'-5'-cyclic adenosine monophosphate (cAMP) and are highly expressed in the brain. Among these receptors, the endogenous expression of GPR3 in cerebellar granule neurons (CGNs) is increas...

journal_title：Neurobiology of disease

authors： Tanaka S,Miyagi T,Dohi E,Seki T,Hide I,Sotomaru Y,Saeki Y,Antonio Chiocca E,Matsumoto M,Sakai N

更新日期：2014-08-01 00:00:00

abstract：:In injury and disease, microglia and astrocytes - two major non-neuronal cell types in the central nervous system (CNS) - undergo morphological, transcriptional, and functional changes, which can underlie pathogenesis and dysfunction of the CNS. Microglia, the brain's tissue resident parenchymal macrophages, are descr...

journal_title：Neurobiology of disease

authors： Bennett ML,Viaene AN

更新日期：2021-01-01 00:00:00

abstract：:Attention deficit hyperactivity disorder (ADHD) is characterized by hyperactivity, inattention, and impulsivity. The coloboma mouse model of ADHD exhibits profound hyperactivity. To determine whether coloboma mice exhibit other signs of ADHD, we assessed latent inhibition as a test of attention, and impulsivity in a d...

journal_title：Neurobiology of disease

authors： Bruno KJ,Freet CS,Twining RC,Egami K,Grigson PS,Hess EJ

更新日期：2007-01-01 00:00:00

abstract：:Glutaric aciduria type I (GA I) is an autosomal recessive inherited metabolic disorder caused by deficiency of glutaryl-CoA dehydrogenase (GCD) resulting in the accumulation of 3-hydroxyglutaric acid (3OHG), glutaric acid and glutaconic acid in body fluids. GA I is characterized by a specific age- and brain region-dep...

journal_title：Neurobiology of disease

authors： Freudenberg F,Lukacs Z,Ullrich K

更新日期：2004-08-01 00:00:00

abstract：:beta-Amyloid is cytotoxic to neurons in culture by increasing hydrogen peroxide and altering calcium homeostasis. We have evaluated the cytotoxicty of beta-amyloid peptides (betaA(25-35) and betaA(1-40)) and generation of hydrogen peroxide on cortical cultured astrocytes. Twenty-four hours after a single addition of e...

journal_title：Neurobiology of disease

authors： Brera B,Serrano A,de Ceballos ML

更新日期：2000-08-01 00:00:00

abstract：:Huntington disease (HD), a neurodegenerative disorder caused by an expanded CAG repeat in the HTT gene, remains without a treatment to modify the course of the illness. Lithium, a drug widely used for the treatment of bipolar disorder, has been shown to exert neuroprotective effects in a number of models of neurologic...

journal_title：Neurobiology of disease

authors： Pouladi MA,Brillaud E,Xie Y,Conforti P,Graham RK,Ehrnhoefer DE,Franciosi S,Zhang W,Poucheret P,Compte E,Maurel JC,Zuccato C,Cattaneo E,Néri C,Hayden MR

更新日期：2012-12-01 00:00:00

abstract：:Charcot-Marie-Tooth disease type 2B (CMT2B) is an inherited axonal peripheral neuropathy. It is characterised by prominent sensory loss, often complicated by severe ulcero-mutilations of toes or feet, and variable motor involvement. Missense mutations in RAB7A, the gene encoding the small GTPase Rab7, cause CMT2B and ...

journal_title：Neurobiology of disease

authors： Janssens K,Goethals S,Atkinson D,Ermanoska B,Fransen E,Jordanova A,Auer-Grumbach M,Asselbergh B,Timmerman V

更新日期：2014-05-01 00:00:00

abstract：:Mutations in the leucine-rich repeat kinase 2 (LRRK2) gene cause late-onset, autosomal dominant Parkinson's disease (PD). The clinical and neurochemical features of LRRK2-linked PD are similar to idiopathic disease although neuropathology is somewhat heterogeneous. Dominant mutations in LRRK2 precipitate neurodegenera...

journal_title：Neurobiology of disease

authors： Tsika E,Kannan M,Foo CS,Dikeman D,Glauser L,Gellhaar S,Galter D,Knott GW,Dawson TM,Dawson VL,Moore DJ

更新日期：2014-11-01 00:00:00

abstract：:Parkinson's disease (PD), characterized by the loss of dopaminergic nigrostriatal projections, is a debilitating neurodegenerative disease which produces bradykinesia, rigidity, tremor and postural instability. The dopamine precursor levodopa (L-Dopa) is the most effective treatment for the amelioration of PD signs an...

journal_title：Neurobiology of disease

authors： Prescott IA,Liu LD,Dostrovsky JO,Hodaie M,Lozano AM,Hutchison WD

更新日期：2014-11-01 00:00:00

abstract：:Posttraumatic epilepsy (PTE) is a major neurodegenerative disease accounting for 20% of symptomatic epilepsy cases. A long latent phase offers a potential window for prophylactic treatment strategies to prevent epilepsy onset, provided that the patients at risk can be identified. Some promising imaging biomarker candi...

journal_title：Neurobiology of disease

authors： Immonen R,Harris NG,Wright D,Johnston L,Manninen E,Smith G,Paydar A,Branch C,Grohn O

更新日期：2019-03-01 00:00:00

abstract：:Amyloid-β is a peptide released by synapses in physiological conditions and its pathological accumulation in brain structures necessary for memory processing represents a key toxic hallmark underlying Alzheimer's disease. The oligomeric form of Amyloid-β (Aβο) is now believed to represent the main Amyloid-β species af...

journal_title：Neurobiology of disease

authors： Kootar S,Frandemiche ML,Dhib G,Mouska X,Lorivel T,Poupon-Silvestre G,Hunt H,Tronche F,Bethus I,Barik J,Marie H

更新日期：2018-10-01 00:00:00

abstract：:Developing in vivo functional and structural neuroimaging assays in Dyt1 ΔGAG heterozygous knock-in (Dyt1 KI) mice provide insight into the pathophysiology underlying DYT1 dystonia. In the current study, we examined in vivo functional connectivity of large-scale cortical and subcortical networks in Dyt1 KI mice and wi...

journal_title：Neurobiology of disease

authors： DeSimone JC,Febo M,Shukla P,Ofori E,Colon-Perez LM,Li Y,Vaillancourt DE

更新日期：2016-11-01 00:00:00