Oxidative stress increases expression and activity of BACE in NT2 neurons.

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：:3-Nitropropionic acid (3-NP) is an irreversible inhibitor of succinate dehydrogenase that has been used to explore the primary mechanisms of cell death associated with mitochondrial dysfunction and neurodegeneration in Huntington's disease. In this study we investigated the ability of brain-derived neurotrophic factor...

journal_title：Neurobiology of disease

authors： Almeida S,Laço M,Cunha-Oliveira T,Oliveira CR,Rego AC

更新日期：2009-09-01 00:00:00

abstract：:Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder that results in motor, cognitive and psychiatric abnormalities. Dysfunction in neuronal processing between the cortex and the basal ganglia is fundamental to the onset and progression of the HD phenotype. The corticosubthalamic hyperdirect p...

journal_title：Neurobiology of disease

authors： Callahan JW,Abercrombie ED

更新日期：2015-06-01 00:00:00

abstract：:Friedreich ataxia (FRDA) is an autosomal recessive neurodegenerative disorder caused by a dynamic GAA repeat expansion mutation within intron 1 of the FXN gene. Studies of mouse models for other trinucleotide repeat (TNR) disorders have revealed an important role of mismatch repair (MMR) proteins in TNR instability. T...

journal_title：Neurobiology of disease

authors： Ezzatizadeh V,Pinto RM,Sandi C,Sandi M,Al-Mahdawi S,Te Riele H,Pook MA

更新日期：2012-04-01 00:00:00

abstract：:Axonal damage, a core feature of neurological diseases, induces a retrograde reaction in neurons and surrounding glia. We determined transcriptional profiles of this reaction using Affymetrix oligonucleotide arrays. Gene expression was examined in spinal cord tissue prior to injury and following ventral root avulsion ...

journal_title：Neurobiology of disease

authors： Swanberg M,Duvefelt K,Diez M,Hillert J,Olsson T,Piehl F,Lidman O

更新日期：2006-10-01 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：:Butyrylcholinesterase (BuChE) is increased in the cerebral cortex of Alzheimer's disease (AD) patients, particularly those carrying epsilon4 allele of the apolipoprotein E gene (ApoE) and certain BuChE variants that predict increased AD risk and poor response to anticholinesterase therapy. We measured BuChE activity a...

journal_title：Neurobiology of disease

authors： Darreh-Shori T,Brimijoin S,Kadir A,Almkvist O,Nordberg A

更新日期：2006-11-01 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：:Microglia and macrophages express the alpha(M)/beta(2) integrin complement-receptor-3 (CR3/MAC-1; CD11b/CD18) and scavenger-receptor-AI/II (SRAI/II). Both can mediate myelin phagocytosis. We document that CR3/MAC-1 mediated myelin phagocytosis in microglia is modulated by complement and anti-CR3/MAC-1 mAbs. Complement...

journal_title：Neurobiology of disease

authors： Reichert F,Rotshenker S

更新日期：2003-02-01 00:00:00

abstract：:Multiple sclerosis (MS) is a chronic, inflammatory autoimmune disease that affects the central nervous system (CNS) for which there is no cure. In MS, encephalitogenic T cells infiltrate the CNS causing demyelination and neuroinflammation; however, little is known about the role of regulatory T cells (Tregs) in CNS ti...

journal_title：Neurobiology of disease

authors： McIntyre LL,Greilach SA,Othy S,Sears-Kraxberger I,Wi B,Ayala-Angulo J,Vu E,Pham Q,Silva J,Dang K,Rezk F,Steward O,Cahalan MD,Lane TE,Walsh CM

更新日期：2020-07-01 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：: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：:The identification of novel molecular targets crucially involved in motor neuron degeneration/survival is a necessary step for the development of hopefully more effective therapeutic strategies for amyotrophic lateral sclerosis (ALS) patients. In this view, S1R, an endoplasmic reticulum (ER)-resident receptor with cha...

journal_title：Neurobiology of disease

authors： Peviani M,Salvaneschi E,Bontempi L,Petese A,Manzo A,Rossi D,Salmona M,Collina S,Bigini P,Curti D

更新日期：2014-02-01 00:00:00

abstract：:Huntingtin, the protein product of the Huntington's disease (HD) gene, is known to interact with the tumor suppressor p53. It has recently been shown that activation of p53 upregulates the level of huntingtin, both in vitro and in vivo, whereas p53 deficiency in HD-transgenic flies and mice has been found to be benefi...

journal_title：Neurobiology of disease

authors： Ryan AB,Zeitlin SO,Scrable H

更新日期：2006-11-01 00:00:00

abstract：:The telomeric copy of the survival motor neuron gene (SMN1) is deleted or mutated in all spinal muscular atrophy (SMA) patients and these patients present mainly a loss in spinal motoneurons. Although studies performed in HeLa cells suggest that SMN may be involved in the biogenesis and possibly in recycling of splice...

journal_title：Neurobiology of disease

authors： Cisterni C,Kallenbach S,Jordier F,Bagnis C,Pettmann B

更新日期：2001-04-01 00:00:00

abstract：:Pituitary adenylate cyclase activating polypeptide (PACAP) and its complementary receptor, PAC1, are crucial in central respiratory control. PACAP Knockout (KO) mice exhibit a SIDS-like phenotype, with an inability to overcome noxious insults, compression of baseline ventilation, and death in the early post-neonatal p...

journal_title：Neurobiology of disease

authors： Huang J,Waters KA,Machaalani R

更新日期：2017-07-01 00:00:00

abstract：:Neural stem cells (NSCs) are multipotent, self-renewing progenitors that generate progeny that differentiate into neurons and glia. NSCs in the adult mammalian brain are generally quiescent. Environmental stimuli such as learning or exercise can activate quiescent NSCs, inducing them to proliferate and produce new neu...

journal_title：Neurobiology of disease

authors： Otsuki L,Brand AH

更新日期：2017-11-01 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：: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：:In the injured central nervous system (CNS), transforming growth factor (TGF)-β1/2-induced scarring and wound cavitation impede axon regeneration implying that a combination of both scar suppression and axogenic treatments is required to achieve functional recovery. After treating acute and chronic dorsal funicular sp...

journal_title：Neurobiology of disease

authors： Ahmed Z,Bansal D,Tizzard K,Surey S,Esmaeili M,Gonzalez AM,Berry M,Logan A

更新日期：2014-04-01 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：:We used quantal dose-titration of a mouse-adapted human transmissible spongiform encephalopathy strain (M470) to compare different analytical methods for their ability to detect asymptomatic brain prion infection after low dose inoculation. At a time point approximately 2.5-fold beyond the mean incubation period of hi...

journal_title：Neurobiology of disease

authors： Collins SJ,Lewis V,Brazier MW,Hill AF,Lawson VA,Klug GM,Masters CL

更新日期：2005-11-01 00:00:00

abstract：:Angelman syndrome (AS) is a neurogenetic disorder caused by loss of maternal UBE3A expression or mutation-induced dysfunction of its protein product, the E3 ubiquitin-protein ligase, UBE3A. In humans and rodents, UBE3A/Ube3a transcript is maternally imprinted in several brain regions, but the distribution of native UB...

journal_title：Neurobiology of disease

authors： Gustin RM,Bichell TJ,Bubser M,Daily J,Filonova I,Mrelashvili D,Deutch AY,Colbran RJ,Weeber EJ,Haas KF

更新日期：2010-09-01 00:00:00

abstract：:We identified a novel spontaneous mutant mouse showing motor symptoms that are similar to those of the dystonia musculorum (dt) mouse. The observations suggested that the mutant mice inherited the mild dt phenotype as an autosomal recessive trait. Linkage analysis showed that the causative gene was located near D1Mit3...

journal_title：Neurobiology of disease

authors： Horie M,Mekada K,Sano H,Kikkawa Y,Chiken S,Someya T,Saito K,Hossain MI,Nameta M,Abe K,Sakimura K,Ono K,Nambu A,Yoshiki A,Takebayashi H

更新日期：2016-12-01 00:00:00

abstract：:Focal mechanical cortical trauma triggers diffuse apoptotic neurodegeneration in the developing rat brain which is associated with invasion of brain tissue with inflammatory mediators. We hypothesized that caspase-1 and the two caspase-1-processed cytokines, interleukin (IL)-1beta and IL-18, are involved in trauma-ind...

journal_title：Neurobiology of disease

authors： Sifringer M,Stefovska V,Endesfelder S,Stahel PF,Genz K,Dzietko M,Ikonomidou C,Felderhoff-Mueser U

更新日期：2007-03-01 00:00:00

abstract：:The use of energy substrates, such as lactate and pyruvate, has been shown to improve synaptic function when administered during glucose deprivation. In the present study, we investigated whether prolonged incubation with monocarboxylate (pyruvate or lactate) prior rather than during glucose deprivation can also susta...

journal_title：Neurobiology of disease

authors： Shetty PK,Sadgrove MP,Galeffi F,Turner DA

更新日期：2012-01-01 00:00:00

abstract：:Blood-brain barrier (BBB) disruption, mediated through matrix metalloproteinases (MMPs) and other mechanisms, is a critical event during ischemic stroke. Tissue plasminogen activator (tPA) is the only FDA-approved thrombolytic therapy for acute ischemic stroke, but the efficacy and safety of its therapeutic applicatio...

journal_title：Neurobiology of disease

authors： Jin R,Yang G,Li G

更新日期：2010-06-01 00:00:00

abstract：:Age-inappropriate expression of juvenile NMDA receptors (NMDARs) containing GluN3A subunits has been linked to synapse loss and death of spiny projection neurons of the striatum (SPNs) in Huntington's disease (HD). Here we show that suppressing GluN3A expression prevents a multivariate synaptic transmission phenotype ...

journal_title：Neurobiology of disease

authors： Mahfooz K,Marco S,Martínez-Turrillas R,Raja MK,Pérez-Otaño I,Wesseling JF

更新日期：2016-09-01 00:00:00

abstract：:Dravet syndrome is a severe infantile-onset epileptic encephalopathy which begins with febrile seizures and is caused by heterozygous loss-of-function mutations of the voltage-gated sodium channel gene SCN1A. We designed a CRISPR-based gene therapy for Scn1a-haplodeficient mice using multiple guide RNAs (gRNAs) in the...

journal_title：Neurobiology of disease

authors： Yamagata T,Raveau M,Kobayashi K,Miyamoto H,Tatsukawa T,Ogiwara I,Itohara S,Hensch TK,Yamakawa K

更新日期：2020-07-01 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