Core features of frontotemporal dementia recapitulated in progranulin knockout mice.

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：: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：:The dystonias are comprised of a group of disorders that share common neurological abnormalities of involuntary twisting or repetitive movements and postures. The most common inherited primary dystonia is DYT1 dystonia, which is due to loss of a GAG codon in the TOR1A gene that encodes torsinA. Autopsy studies of brai...

journal_title：Neurobiology of disease

authors： Song CH,Bernhard D,Bolarinwa C,Hess EJ,Smith Y,Jinnah HA

更新日期：2013-06-01 00:00:00

abstract：:Alzheimer's disease is widely held to be associated with oxidative stress due, in part, to the membrane action of amyloid beta-peptide (A beta) aggregates. In this study, the involvement of oxidative stress on A beta-induced energy metabolism dysfunction was evaluated on PC12 cells. It was shown that A beta peptides (...

journal_title：Neurobiology of disease

authors： Pereira C,Santos MS,Oliveira C

更新日期：1999-06-01 00:00:00

abstract：:Increasing evidence has linked inflammatory processes to neurodegenerative disorders, including Alzheimer's and Parkinson's disease (PD). Tumor necrosis factor alpha (TNF-alpha) is a key inflammatory cytokine and several studies linked increased TNF-alpha to dopaminergic cell death in PD. The TNF-alpha promoter sequen...

journal_title：Neurobiology of disease

authors： Pieper HC,Evert BO,Kaut O,Riederer PF,Waha A,Wüllner U

更新日期：2008-12-01 00:00:00

abstract：:Over 1250 mutations in SCN1A, the Nav1.1 voltage-gated sodium channel gene, are associated with seizure disorders including GEFS+. To evaluate how a specific mutation, independent of genetic background, causes seizure activity we generated two pairs of isogenic human iPSC lines by CRISPR/Cas9 gene editing. One pair is...

journal_title：Neurobiology of disease

authors： Xie Y,Ng NN,Safrina OS,Ramos CM,Ess KC,Schwartz PH,Smith MA,O'Dowd DK

更新日期：2020-02-01 00:00:00

abstract：:Mesenchymal stem cells (MSCs) are reported to possess immunomodulatory properties. Previous reports have demonstrated the beneficial effects of MSC-transplantation in focal cerebral ischemia animal models. In this study, we have investigated the neuroimmunomodulatory functions of human MSCs, transplanted in a rat foca...

journal_title：Neurobiology of disease

authors： Sheikh AM,Nagai A,Wakabayashi K,Narantuya D,Kobayashi S,Yamaguchi S,Kim SU

更新日期：2011-03-01 00:00:00

abstract：:Neurofibrillary degeneration in transgenic models of tauopathies has been observed to be enhanced when these models are crossed with transgenic models developing an Aβ pathology. The mechanisms leading to this enhanced tau pathology are not well understood. We have performed a detailed analysis of tau misprocessing in...

journal_title：Neurobiology of disease

authors： Héraud C,Goufak D,Ando K,Leroy K,Suain V,Yilmaz Z,De Decker R,Authelet M,Laporte V,Octave JN,Brion JP

更新日期：2014-02-01 00:00:00

abstract：:Heterozygous in frame duplications of the PHOX2B gene, leading to polyalanine (polyAla) expansions ranging from +5 to +13 residues of a 20-alanine stretch, have been identified in the vast majority of patients affected with Congenital Central Hypoventilation Syndrome (CCHS), a rare neurocristopathy characterized by ab...

journal_title：Neurobiology of disease

authors： Di Zanni E,Bachetti T,Parodi S,Bocca P,Prigione I,Di Lascio S,Fornasari D,Ravazzolo R,Ceccherini I

更新日期：2012-01-01 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：: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：:Beta power suppression in the basal ganglia is stronger during movements that require high force levels and high movement effort but it has been difficult to dissociate the two. We recorded scalp EEG and basal ganglia local field potentials in Parkinson's disease patients (11 STN, 7 GPi) ON and OFF dopaminergic medica...

journal_title：Neurobiology of disease

authors： Fischer P,Pogosyan A,Green AL,Aziz TZ,Hyam J,Foltynie T,Limousin P,Zrinzo L,Samuel M,Ashkan K,Da Lio M,De Cecco M,Fornaser A,Brown P,Tan H

更新日期：2019-07-01 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：: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：:This article reviews three concepts related to implantable brain computer interface (BCI) devices being designed for human use: neural signal extraction primarily for motor commands, signal insertion to restore sensation, and technological challenges that remain. A significant body of literature has occurred over the ...

journal_title：Neurobiology of disease

authors： Konrad P,Shanks T

更新日期：2010-06-01 00:00:00

abstract：:The critical anabolic and trophic role of signaling by insulin-like growth factors (IGF) I and II via the type-I IGF receptor (IGF-IR) is reviewed throughout the life of skeletal myocytes. The proliferative effects of IGF-IR stimulation, both during embryogenesis and during satellite cell proliferation following dener...

journal_title：Neurobiology of disease

authors： Singleton JR,Feldman EL

更新日期：2001-08-01 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：: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：:The clinical trials employing neuroprotectants targeting single, early pathogenic mechanisms in stroke have so far been barely successful. We found in human postmortem stroke brains that in addition to apoptosis, necroptosis also contributed to neuronal damage. Thus, a new strategy targeting both mechanisms might be n...

journal_title：Neurobiology of disease

authors： Han F,Guan X,Guo W,Lu B

更新日期：2019-07-01 00:00:00

abstract：:The SMN2 transgenic mouse, Tg(SMN2)89Ahmb, has emerged as the most widely used in spinal muscular atrophy (SMA) research. Here we clone the genomic integration site of the transgene and demonstrate it to be in intron 4 of the metabotropic glutamate receptor 7 (mGluR7) gene. We found that the integration of this transg...

journal_title：Neurobiology of disease

authors： Gogliotti RG,Lutz C,Jorgensen M,Huebsch K,Koh S,Didonato CJ

更新日期：2011-07-01 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：:It is well established that the conversion of PrP(C) to PrP(Sc) is the key event in prion disease biology. In addition, several lines of evidence suggest that glycosaminoglycans (GAGs) and in particular heparan sulfate (HS) may play a role in the PrP(C) to PrP(Sc) conversion process. It has been proposed that PrP(Sc) ...

journal_title：Neurobiology of disease

authors： Mayer-Sonnenfeld T,Zeigler M,Halimi M,Dayan Y,Herzog C,Lasmezas CI,Gabizon R

更新日期：2005-12-01 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：:Parkinson's disease is a neurodegenerative disorder characterized by the progressive loss of dopaminergic neurons in the substantia nigra and a marked reduction of dopamine (DA) levels in the striatum. Binding to its specific receptors, DA switches on a complex program of intracellular signaling that regulates gene ex...

journal_title：Neurobiology of disease

authors： Cuadrado-Tejedor M,Sesma MT,Giménez-Amaya JM,Ortiz L

更新日期：2005-12-01 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：:Environmental stress is among the most important contributors to increased susceptibility to develop psychiatric disorders, including anxiety and post-traumatic stress disorder. While even acute stress alters gene expression, the molecular mechanisms underlying these changes remain largely unknown. 5-hydroxymethylcyto...

journal_title：Neurobiology of disease

authors： Li S,Papale LA,Zhang Q,Madrid A,Chen L,Chopra P,Keleş S,Jin P,Alisch RS

更新日期：2016-02-01 00:00:00

abstract：:A crucial question in polyQ-induced neurodegeneration is the influence of wild type protein on the formation of aggregates and toxicity. Recently it was shown that non-expanded ataxin-3 protein mitigated neurodegeneration in a Drosophila and mouse model of SCA3. We now explored the effects of overexpressing non-expand...

journal_title：Neurobiology of disease

authors： Hübener J,Riess O

更新日期：2010-04-01 00:00:00

abstract：:Schizophrenia is a complex neuropsychiatric disorder that involves disturbances in neural circuitry and synaptic function. The exquisite network architecture and capacity for discreet post-synaptic remodeling of neurons requires coordination by an elaborate intracellular network of molecular signal transduction system...

journal_title：Neurobiology of disease

authors： Beveridge NJ,Cairns MJ

更新日期：2012-05-01 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：:MicroRNAs (miRNA), a class of non-coding RNAs, are emerging as important modulators of neuronal development, structure and function. A connection has been established between abnormalities in miRNA expression and miRNA-mediated gene regulation and psychiatric and neurodevelopmental disorders as well as cognitive dysfu...

journal_title：Neurobiology of disease

authors： Xu B,Hsu PK,Karayiorgou M,Gogos JA

更新日期：2012-05-01 00:00:00