abstract：:Amino-terminal fragments of huntingtin (htt) appear to result from proteolytic processing of the full-length protein in Huntington's disease (HD), and fragments containing pathological expansions of polyglutamine elicit toxicity in model systems. Such fragments are sequestered into insoluble aggregates, which may init...

journal_title：Neurobiology of disease

authors： Miller TW,Zhou C,Gines S,MacDonald ME,Mazarakis ND,Bates GP,Huston JS,Messer A

更新日期：2005-06-01 00:00:00

abstract：:The activation of soluble guanylate cyclase by nitric oxide is increased in the frontal cortex but is reduced in the cerebellum of patients who died with liver cirrhosis. The aims of this work were to assess whether hyperammonemia is responsible for the region-selective alterations in guanylate cyclase modulation in l...

journal_title：Neurobiology of disease

authors： Rodrigo R,Erceg S,Felipo V

更新日期：2005-06-01 00:00:00

abstract：:The brain of Alzheimer's disease patients shows abundant dystrophic neurites in close proximity to fibrillar beta-amyloid (A beta) plaques, and activated glial cells. We evaluated the influence of pro-inflammatory molecules (LPS + IFN-gamma) on A beta(1-42) neurotoxicity. 2 microM A beta(1-42) induced apoptosis of hip...

journal_title：Neurobiology of disease

authors： Ramírez G,Toro R,Döbeli H,von Bernhardi R

更新日期：2005-06-01 00:00:00

abstract：:Amyotrophic lateral sclerosis (ALS) is characterized by a progressive loss of large motor neurons in the brain and spinal cord. Amyloid precursor protein (APP), the transmembrane precursor of beta-amyloid (A beta), accumulates in the anterior horn motor neurons of ALS patients with mild lesions. APP undergoes an alter...

journal_title：Neurobiology of disease

authors： Calingasan NY,Chen J,Kiaei M,Beal MF

更新日期：2005-06-01 00:00:00

abstract：:Converging lines of evidence suggest that neuroinflammatory processes may account for the progressive death of dopaminergic neurons in Parkinson's disease (PD). Therefore, anti-inflammatory strategies have attracted much interest for their potential to prevent further deterioration of PD. Our previous study showed tha...

journal_title：Neurobiology of disease

authors： Zhou HF,Liu XY,Niu DB,Li FQ,He QH,Wang XM

更新日期：2005-04-01 00:00:00

abstract：:Oculopharyngeal muscular dystrophy (OPMD) is a late-onset disorder caused by a (GCG)n trinucleotide repeat expansion in the poly(A) binding protein nuclear-1 (PABPN1) gene, which in turn leads to an expanded polyalanine tract in the protein. We generated transgenic mice expressing either the wild type or the expanded ...

journal_title：Neurobiology of disease

authors： Dion P,Shanmugam V,Gaspar C,Messaed C,Meijer I,Toulouse A,Laganiere J,Roussel J,Rochefort D,Laganiere S,Allen C,Karpati G,Bouchard JP,Brais B,Rouleau GA

更新日期：2005-04-01 00:00:00

abstract：:In addition to the substantial biological diversity among humans, our limited ability to reliably measure expression changes of small magnitude significantly reduces our capacity to obtain convergent sets of transcriptome data in postmortem brain. In particular, differences in the structure and sensitivity/reproducibi...

journal_title：Neurobiology of disease

authors： Hollingshead D,Lewis DA,Mirnics K

更新日期：2005-04-01 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：:Peripherin is a type III intermediate filament protein normally undetectable in most brain neurons. Here, we report a similar pattern of peripherin expression in the brains of both mice treated with systemic injections of kainic acid (KA) and in peripherin transgenic mice (Per mice) over-expressing the normal peripher...

journal_title：Neurobiology of disease

authors： Kriz J,Beaulieu JM,Julien JP,Krnjević K

更新日期：2005-03-01 00:00:00

abstract：:Extracellular nucleotides exert a variety of biological actions through different subtypes of P2 receptors. Here we characterized in the human neuroblastoma SH-SY5Y cells the simultaneous presence of various P2 receptors, belonging to the P2X ionotropic and P2Y metabotropic families. Western blot analysis detected the...

journal_title：Neurobiology of disease

authors： Cavaliere F,Nestola V,Amadio S,D'Ambrosi N,Angelini DF,Sancesario G,Bernardi G,Volonté C

更新日期：2005-02-01 00:00:00

abstract：:Intraneocortical injection of ibotenate, a glutamate analog, in newborn mice produces damage mimicking lesions observed in human infants with cerebral palsy. Previous research using this model has demonstrated that pretreatment with IL-9, a Th2 cytokine, significantly exacerbated excitotoxic brain lesions. The goal of...

journal_title：Neurobiology of disease

authors： Mesplès B,Fontaine RH,Lelièvre V,Launay JM,Gressens P

更新日期：2005-02-01 00:00:00

abstract：:The worldwide proliferation of mobile phones raises the question of the effects of 900-MHz electromagnetic fields (EMF) on the brain. Using a head-only exposure device in the rat, we showed that a 15-min exposure to 900-MHz pulsed microwaves at a high brain-averaged power of 6 W/kg induced a strong glial reaction in t...

journal_title：Neurobiology of disease

authors： Mausset-Bonnefont AL,Hirbec H,Bonnefont X,Privat A,Vignon J,de Sèze R

更新日期：2004-12-01 00:00:00

abstract：:Identified as the cause of some familial forms of Parkinson disease (PD) and as one of the major component of Lewy bodies, alpha-synuclein (alpha-syn) became the molecular hallmark of several neurodegenerative conditions now designated as synucleinopathies. Transgenic models have been generated to elucidate its physio...

journal_title：Neurobiology of disease

authors： Fernagut PO,Chesselet MF

更新日期：2004-11-01 00:00:00

abstract：:We have examined the pattern of striatal messenger RNA expression of over 8000 genes in a rat model of levodopa (L-DOPA)-induced dyskinesia and Parkinson disease (PD). 6-Hydroxydopamine (6-OHDA)-lesioned rats were treated with L-DOPA or physiological saline for 22 days and repeatedly tested for antiakinetic response t...

journal_title：Neurobiology of disease

authors： Konradi C,Westin JE,Carta M,Eaton ME,Kuter K,Dekundy A,Lundblad M,Cenci MA

更新日期：2004-11-01 00:00:00

abstract：:The deposition and accumulation of amyloid-beta-peptide (Abeta) in the brain are considered a sine qua non for Alzheimer's disease. The experimental delivery of fibrilized Abeta serves as a cellular model for several facets of the disease including the induction of synaptic dysfunction and apoptosis. c-Abl kinase is i...

journal_title：Neurobiology of disease

authors： Alvarez AR,Sandoval PC,Leal NR,Castro PU,Kosik KS

更新日期：2004-11-01 00:00:00

abstract：:Microglial cells involved in the pathogenesis of many neurodegenerative diseases acquire the features of cytotoxic and phagocytic cells in response to certain pathogens and inflammatory signals. K(ATP) channels are energy sensors of ATP availability that link the cell's metabolic state to its membrane excitability. In...

journal_title：Neurobiology of disease

authors： Ramonet D,Rodríguez MJ,Pugliese M,Mahy N

更新日期：2004-10-01 00:00:00

abstract：:We have explored the molecular mechanism underlying amyloid beta-peptide (Abeta)-mediated cytotoxicity in vitro. Exposure of murine cerebral endothelial cells (CECs) or C6 glioma cells to Abeta25-35 resulted in dose-dependent cell death. Ceramide is a pro-apoptotic lipid mediator. Forced elevation of cellular ceramide...

journal_title：Neurobiology of disease

authors： Yang DI,Yeh CH,Chen S,Xu J,Hsu CY

更新日期：2004-10-01 00:00:00

abstract：:Uridine, like adenosine, is released under sustained depolarization and it can inhibit hippocampal neuronal activity, suggesting that uridine may be released during seizures and can be involved in epileptic mechanisms. In an in vivo microdialysis study, we measured the extracellular changes of nucleoside and amino aci...

journal_title：Neurobiology of disease

authors： Slézia A,Kékesi AK,Szikra T,Papp AM,Nagy K,Szente M,Maglóczky Z,Freund TF,Juhász G

更新日期：2004-08-01 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：:The mutilated-foot rat (mf rat) is an autosomal recessive mutant with characteristic digit deformities in adult animals, and this phenotype mimics many aspects of human sensory neuropathy. The genetics of mf rats was recently elucidated. To understand whether the genotype is responsible for cutaneous denervation befor...

journal_title：Neurobiology of disease

authors： Hsu SH,Lee MJ,Hsieh SC,Scaravilli F,Hsieh ST

更新日期：2004-07-01 00:00:00

abstract：:The developing rodent brain is vulnerable to pharmacological blockade of N-methyl-d-aspartate (NMDA) receptors which can lead to severe and disseminated apoptotic neurodegeneration. Here, we show that systemic administration of the NMDA receptor antagonist MK801 to 7-day-old rats leads to impaired activity of extracel...

journal_title：Neurobiology of disease

authors： Hansen HH,Briem T,Dzietko M,Sifringer M,Voss A,Rzeski W,Zdzisinska B,Thor F,Heumann R,Stepulak A,Bittigau P,Ikonomidou C

更新日期：2004-07-01 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：:Tumor necrosis factor-alpha (TNF-alpha) is critically involved in inflammation and may participate in hippocampal injury in bacterial meningitis. In a mouse model of ceftriaxone-treated pneumococcal meningitis, spatial memory and motor performance of TNF-alpha-deficient (n = 57) and control mice (n = 55) were investig...

journal_title：Neurobiology of disease

authors： Gerber J,Böttcher T,Hahn M,Siemer A,Bunkowski S,Nau R

更新日期：2004-06-01 00:00:00

abstract：:Dystonias are movement disorders whose pathomechanism is largely unknown. Dystonic dt(sz) hamsters represent a model of primary dystonias, where alterations of striatal interneuron density and sodium channel function in projection neurones were described. Here, using cortico-striatal slices, we explore whether also th...

journal_title：Neurobiology of disease

authors： Köhling R,Koch UR,Hamann M,Richter A

更新日期：2004-06-01 00:00:00

abstract：:Spinal cord injury (SCI) is a major cause of disability, and at present, there is no universally accepted treatment. The functional decline following SCI is contributed to both direct mechanical injury and secondary pathophysiological mechanisms that are induced by the initial trauma. These mechanisms initially involv...

journal_title：Neurobiology of disease

authors： Profyris C,Cheema SS,Zang D,Azari MF,Boyle K,Petratos S

更新日期：2004-04-01 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：:Markers of the kynurenine pathway were studied in postmortem frontal cortex obtained from individuals with schizophrenia and controls. Quantitative endpoint RT-PCR was used to measure mRNA transcripts. Of the two enzymes capable of catalyzing the first step in the pathway, tryptophan 2,3-dioxygenase (TDO2) and indolea...

journal_title：Neurobiology of disease

authors： Miller CL,Llenos IC,Dulay JR,Barillo MM,Yolken RH,Weis S

更新日期：2004-04-01 00:00:00

abstract：:Rett syndrome (RS) is caused by mutations in the gene encoding methyl-CpG-binding protein 2 (MeCP2) and is characterized by arrested postnatal neurodevelopment. We followed the expression of MeCP2 protein in various brain structures of normal rat from birth to 2 years of age. By measuring the amount of protein using t...

journal_title：Neurobiology of disease

authors： Cassel S,Revel MO,Kelche C,Zwiller J

更新日期：2004-03-01 00:00:00

abstract：:Frontotemporal dementia (FTD) is the second most common type of primary degenerative dementia. Some patients present an overlap between Alzheimer's disease (AD) and FTD both in neuropathological and clinical aspects. This may suggest a similar overlap in physiopathology, namely an involvement of mitochondrial DNA (mtD...

journal_title：Neurobiology of disease

authors： Grazina M,Silva F,Santana I,Santiago B,Mendes C,Simões M,Oliveira M,Cunha L,Oliveira C

更新日期：2004-03-01 00:00:00

abstract：:Rubrospinal neurons (RSNs) undergo marked atrophy after cervical axotomy. This progressive atrophy may impair the regenerative capacity of RSNs in response to repair strategies that are targeted to promote rubrospinal tract regeneration. Here, we investigated whether we could achieve long-term rescue of RSNs from lesi...

journal_title：Neurobiology of disease

authors： Ruitenberg MJ,Blits B,Dijkhuizen PA,te Beek ET,Bakker A,van Heerikhuize JJ,Pool CW,Hermens WT,Boer GJ,Verhaagen J

更新日期：2004-03-01 00:00:00

abstract：:To study the role of Abeta amyloid deposits in the generation of cytoskeletal lesions, we have generated a transgenic mouse line coexpressing in the same neurons a wild-type human tau isoform (0N3R), a mutant form of APP (751SL) and a mutant form of PS1 (M146L). These mice developed early cerebral extracellular deposi...

journal_title：Neurobiology of disease

authors： Boutajangout A,Authelet M,Blanchard V,Touchet N,Tremp G,Pradier L,Brion JP

更新日期：2004-02-01 00:00:00

abstract：:An important aspect of Huntington's disease (HD) pathogenesis which may have important therapeutic implications is that the cellular events leading to cell death may be different in cortical and striatal neurons. In the present study, we characterized cellular changes in cortical and striatal neurons treated with the ...

journal_title：Neurobiology of disease

authors： Galas MC,Bizat N,Cuvelier L,Bantubungi K,Brouillet E,Schiffmann SN,Blum D

更新日期：2004-02-01 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：: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：:Previous studies have indicated that administration of glial cell line-derived neurotrophic factor (GDNF) counteracts neuronal death after stroke. However, in these studies damage was evaluated at most a few days after the insult. Here, we have explored the long-term consequences of two routes of GDNF delivery to the ...

journal_title：Neurobiology of disease

authors： Arvidsson A,Kirik D,Lundberg C,Mandel RJ,Andsberg G,Kokaia Z,Lindvall O

更新日期：2003-12-01 00:00:00

abstract：:Shed photoreceptor outer segments (POS) are phagocytosed by RPE cells in a circadian manner. The homozygous deletion of the c-mer gene abolishes the ingestion phase of this phagocytosis in the Royal College of Surgeons (RCS) rat strain, which in turn leads to the death of photoreceptor cells. We identified RPE transcr...

journal_title：Neurobiology of disease

authors： Dufour EM,Nandrot E,Marchant D,Van Den Berghe L,Gadin S,Issilame M,Dufier JL,Marsac C,Carper D,Menasche M,Abitbol M

更新日期：2003-11-01 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：:Senile plaques composed mainly of beta-amyloid (Abeta) and neurofibrillary tangles principally composed of hyperphosphorylated tau are the major pathological features of Alzheimer's disease (AD). Despite the fact that increased expression of amyloid precursor protein (APP) and presenilin-1 (PS1) transgenes in mice lea...

journal_title：Neurobiology of disease

authors： Kurt MA,Davies DC,Kidd M,Duff K,Howlett DR

更新日期：2003-10-01 00:00:00

abstract：:Failure of cell cycle regulation in neurons might be critically involved in the process of neurodegeneration in Alzheimer's disease (AD). We present here evidence to support the hypothesis that cell cycle alterations occur in cells other than neurons in AD sufferers. Lymphocytes from AD patients immortalized with Epst...

journal_title：Neurobiology of disease

authors： de las Cuevas N,Urcelay E,Hermida OG,Saíz-Diaz RA,Bermejo F,Ayuso MS,Martín-Requero A

更新日期：2003-08-01 00:00:00

abstract：:We determined whether the preferential toxicity of tetrahydrobiopterin (BH4) on dopamine-producing cells, which we have previously observed in vitro, might also occur in vivo and generate characteristics associated with Parkinson's disease. Intrastriatal BH4 injection caused a loss of tyrosine hydroxylase immunoreacti...

journal_title：Neurobiology of disease

authors： Kim SW,Jang YJ,Chang JW,Hwang O

更新日期：2003-07-01 00:00:00