Characterization of a commonly used mouse model of SMA reveals increased seizure susceptibility and heightened fear response in FVB/N mice.

abstract：:Freezing of gait (FOG) is a devastating axial motor symptom in Parkinson's disease (PD) leading to falls, institutionalization, and even death. The response of FOG to dopaminergic medication and deep brain stimulation (DBS) is complex, variable, and yet to be optimized. Fundamental gaps in the knowledge of the underly...

journal_title：Neurobiology of disease

authors： Anidi C,O'Day JJ,Anderson RW,Afzal MF,Syrkin-Nikolau J,Velisar A,Bronte-Stewart HM

更新日期：2018-12-01 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：: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：:The dysregulation of glycogen synthase kinase-3 (GSK3) has been implicated in Alzheimer disease (AD) pathogenesis and in Abeta-induced neurotoxicity, leading us to investigate it as a therapeutic target in an intracerebroventricular Abeta infusion model. Infusion of a specific GSK3 inhibitor SB216763 (SB) reduced a do...

journal_title：Neurobiology of disease

authors： Hu S,Begum AN,Jones MR,Oh MS,Beech WK,Beech BH,Yang F,Chen P,Ubeda OJ,Kim PC,Davies P,Ma Q,Cole GM,Frautschy SA

更新日期：2009-02-01 00:00:00

abstract：:NMDA receptor dysfunction is central to the encephalopathies caused by missense mutations in the NMDA receptor subunit genes. Missense variants of GRIN1, GRIN2A, and GRIN2B cause similar syndromes with varying severity of intellectual impairment, autism, epilepsy, and motor dysfunction. To gain insight into possible b...

journal_title：Neurobiology of disease

authors： Intson K,van Eede MC,Islam R,Milenkovic M,Yan Y,Salahpour A,Henkelman RM,Ramsey AJ

更新日期：2019-12-01 00:00:00

abstract：:Proteolytic cleavage of the amyloid precursor protein (APP) by the two proteases α- and β-secretases controls the generation of the amyloid β peptide (Aβ), a key player in Alzheimer's disease pathogenesis. The α-secretase ADAM10 and the β-secretase BACE1 have opposite effects on Aβ generation and are assumed to compet...

journal_title：Neurobiology of disease

authors： Colombo A,Wang H,Kuhn PH,Page R,Kremmer E,Dempsey PJ,Crawford HC,Lichtenthaler SF

更新日期：2013-01-01 00:00:00

abstract：:The amyloid precursor protein (APP) is a type I transmembrane protein translocated to neuronal terminals, whose function is still unknown. The C-terminus of APP mediates its interaction with cellular adaptor and signaling proteins, some of which signal to the stress-activated protein kinase (SAPK) pathway. Here we sho...

journal_title：Neurobiology of disease

authors： Galvan V,Banwait S,Spilman P,Gorostiza OF,Peel A,Ataie M,Crippen D,Huang W,Sidhu G,Ichijo H,Bredesen DE

更新日期：2007-10-01 00:00:00

abstract：:Several reports indicate that the activity of the hypothalamic–pituitary–adrenal axis (HPA) is increased after a brain insult and that its down-regulation can improve detrimental outcomes associated with ischemic brain injuries.Granulocyte-colony stimulating factor (G-CSF) is a neuroprotective drug shown in the naïve ...

journal_title：Neurobiology of disease

authors： Charles MS,Ostrowski RP,Manaenko A,Duris K,Zhang JH,Tang J

更新日期：2012-07-01 00:00:00

abstract：:Several lines of evidence indicate that glutamate plays a crucial role in the initiation of seizures and their propagation; abnormal glutamate release causes synchronous firing of large populations of neurons, leading to seizures. In the present study, we investigated whether enhanced glutamate uptake by increased gli...

journal_title：Neurobiology of disease

authors： Kong Q,Takahashi K,Schulte D,Stouffer N,Lin Y,Lin CL

更新日期：2012-08-01 00:00:00

abstract：:T cells are believed to be key effector cells in multiple sclerosis (MS). In this study, we examined the roles of T cell ephrinB1 (EFNB1) and ephrinB2 (EFNB2) in the pathogenesis of experimental autoimmune encephalomyelitis (EAE) and MS. We provide evidence that animals with T cell specific double deletion of EFNB1 an...

journal_title：Neurobiology of disease

authors： Luo H,Broux B,Wang X,Hu Y,Ghannam S,Jin W,Larochelle C,Prat A,Wu J

更新日期：2016-07-01 00:00:00

abstract：:Deletion of the mTOR pathway inhibitor PTEN from postnatally-generated hippocampal dentate granule cells causes epilepsy. Here, we conducted field potential, whole cell recording and single cell morphology studies to begin to elucidate the mechanisms by which granule cell-specific PTEN-loss produces disease. Cells fro...

journal_title：Neurobiology of disease

authors： Santos VR,Pun RYK,Arafa SR,LaSarge CL,Rowley S,Khademi S,Bouley T,Holland KD,Garcia-Cairasco N,Danzer SC

更新日期：2017-12-01 00:00:00

abstract：:Cellular mechanisms play a role in conversion of the normal prion protein PrP(C) to the disease-associated protein PrP(Sc). The cells provide not only PrP(C), but also still largely undefined factors required for efficient prion replication. Previously, we have observed that interference with ERK and p38-JNK MAP kinas...

journal_title：Neurobiology of disease

authors： Allard EK,Grujic M,Fisone G,Kristensson K

更新日期：2013-10-01 00:00:00

abstract：:We determined whether tetrahydrobiopterin(BH4), an endogenous cofactor for dopamine(DA) synthesis, causes preferential damage to DArgic neurons among primary cultured rat mesencephalic neurons and whether the death mechanism has relevance to Parkinson's disease (PD). DArgic neurons were more vulnerable to BH4 than non...

journal_title：Neurobiology of disease

authors： Lee SY,Moon Y,Hee Choi D,Jin Choi H,Hwang O

更新日期：2007-01-01 00:00:00

abstract：:Males and females show a different predisposition to certain types of seizures in clinical studies. Animal studies have provided growing evidence for sexual dimorphism of certain brain regions, including those that control seizures. Seizures are modulated by networks involving subcortical structures, including thalamu...

journal_title：Neurobiology of disease

authors： Giorgi FS,Galanopoulou AS,Moshé SL

更新日期：2014-12-01 00:00:00

abstract：:In the present study we analyze the molecular mechanisms underlying motor neuron degeneration in familial amyotrophic lateral sclerosis (FALS). For this, we used a transgenic mouse model expressing the Cu/Zn superoxide dismutase (SOD1) gene with a Gly(86) to Arg (G86R) mutation equivalent to that found in a subset of ...

journal_title：Neurobiology of disease

authors： Dupuis L,de Tapia M,René F,Lutz-Bucher B,Gordon JW,Mercken L,Pradier L,Loeffler JP

更新日期：2000-08-01 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：:Neurodegeneration is a common neuropathological feature of prion diseases. Although evidence of apoptosis was found in natural and experimental prion diseases, the precise mechanisms by which neurons die are poorly understood. The pro-apoptotic BAX protein, a key factor of the mitochondrial pathway, plays a central ro...

journal_title：Neurobiology of disease

authors： Coulpier M,Messiaen S,Hamel R,Fernández de Marco M,Lilin T,Eloit M

更新日期：2006-09-01 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：: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：:Glutathione (GSH) is an important neuroprotective molecule in the brain. The strategy to increase neuronal GSH level is a promising approach to the treatment of neurodegenerative diseases. However, the regulatory mechanism by which neuron-specific GSH synthesis is facilitated remains elusive. Glutamate transporter-ass...

journal_title：Neurobiology of disease

authors： Aoyama K,Wang F,Matsumura N,Kiyonari H,Shioi G,Tanaka K,Kinoshita C,Kikuchi-Utsumi K,Watabe M,Nakaki T

更新日期：2012-03-01 00:00:00

abstract：:The motor cortex and subthalamic nucleus (STN) of patients with Parkinson's disease (PD) exhibit abnormally high levels of electrophysiological oscillations in the ~12-35 Hz beta-frequency range. Recent studies have shown that beta is partly carried forward to regulate future motor states in the healthy condition, sug...

journal_title：Neurobiology of disease

authors： Polar CA,Gupta R,Lehmkuhle MJ,Dorval AD

更新日期：2018-09-01 00:00:00

abstract：:Immunization of mouse models of Alzheimer disease (AD) with amyloid-peptide (Abeta) reduces Abeta deposits and attenuates their memory and learning deficits. Recent clinical trials were halted due to meningoencephalitis, presumably induced by T cell mediated and/or Fc-mediated immune responses. Because injection of an...

journal_title：Neurobiology of disease

authors： Fukuchi K,Tahara K,Kim HD,Maxwell JA,Lewis TL,Accavitti-Loper MA,Kim H,Ponnazhagan S,Lalonde R

更新日期：2006-09-01 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 a fatal neurodegenerative disorder caused by an expanded CAG repeat. Its length can be used to estimate the time of clinical diagnosis, which is defined by overt motor symptoms. Non-motor symptoms begin before motor onset, and involve changes in hypothalamus-regulated functions such as sleep...

journal_title：Neurobiology of disease

authors： Soneson C,Fontes M,Zhou Y,Denisov V,Paulsen JS,Kirik D,Petersén A,Huntington Study Group PREDICT-HD investigators.

更新日期：2010-12-01 00:00:00

abstract：:Inflammation contributes to ischemic brain injury. However, translation of experimental findings from animal models into clinical trials is still ineffective, since the majority of human stroke studies mainly focus on acute neuroprotection, thereby neglecting inflammatory mechanisms and inflammation-associated co-morb...

journal_title：Neurobiology of disease

authors： Herz J,Hagen SI,Bergmüller E,Sabellek P,Göthert JR,Buer J,Hansen W,Hermann DM,Doeppner TR

更新日期：2014-02-01 00:00:00

abstract：:Rare de novo mutations in genes associated with inherited Mendelian disorders are potential contributors to sporadic disease. DYT1 dystonia is an autosomal dominant, early-onset, generalized dystonia associated with an in-frame, trinucleotide deletion (n. delGAG, p. ΔE 302/303) in the Tor1a gene. Here we examine the s...

journal_title：Neurobiology of disease

authors： Bhagat SL,Qiu S,Caffall ZF,Wan Y,Pan Y,Rodriguiz RM,Wetsel WC,Badea A,Hochgeschwender U,Calakos N

更新日期：2016-09-01 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：:Epileptic activity may be more prevalent in early stage Alzheimer's disease (AD) than previously believed. Several studies report spontaneous seizures and interictal discharges in mouse models of AD undergoing age-related Aβ accumulation. The mechanism by which Aβ-induced neuronal excitability can trigger epileptiform...

journal_title：Neurobiology of disease

authors： Lei M,Xu H,Li Z,Wang Z,O'Malley TT,Zhang D,Walsh DM,Xu P,Selkoe DJ,Li S

更新日期：2016-01-01 00:00:00

abstract：:Transient receptor potential vanilloid 1 (TRPV1) channels are involved in several inflammatory diseases. However, their action is still controversial, and both pro-inflammatory and anti-inflammatory roles have been described. We used a strain of TRPV1-KO mice to characterize the role of these channels in experimental ...

journal_title：Neurobiology of disease

authors： Musumeci G,Grasselli G,Rossi S,De Chiara V,Musella A,Motta C,Studer V,Bernardi G,Haji N,Sepman H,Fresegna D,Maccarrone M,Mandolesi G,Centonze D

更新日期：2011-09-01 00:00:00

abstract：:Although the incidence of seizures after a cerebrovascular event including intracerebral hemorrhage has been widely recognized, the present studies have demonstrated that generalized convulsive seizures can cause multifocal amygdaloallocortical hemorrhage and tissue necrosis, the origin of which remains to be establis...

journal_title：Neurobiology of disease

authors： Mraovitch S,Calando Y,Régnier A,Lamproglou I,Vicaut E

更新日期：2005-08-01 00:00:00