abstract：:Human apolipoprotein E (apoE) exists in three isoforms: apoE2, apoE3 and apoE4. APOE ε4 is a major genetic risk factor for cardiovascular disease (CVD) and Alzheimer's disease (AD). ApoE mediates cholesterol metabolism by binding various receptors. The low-density lipoprotein receptor (LDLR) has a high affinity for ap...

journal_title：Neurobiology of disease

authors： Johnson LA,Olsen RH,Merkens LS,DeBarber A,Steiner RD,Sullivan PM,Maeda N,Raber J

更新日期：2014-04-01 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：:Multiple system atrophy is a progressive, neurodegenerative disease characterized by parkinsonism, ataxia, autonomic dysfunction, and accumulation of α-synuclein in oligodendrocytes. To understand how α-synuclein aggregates impact oligodendroglial homeostasis, we investigated an oligodendroglial cell model of α-synucl...

journal_title：Neurobiology of disease

authors： Kragh CL,Gysbers AM,Rockenstein E,Murphy K,Halliday GM,Masliah E,Jensen PH

更新日期：2014-03-01 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：: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：:Since aberrant miRNA expression has been implicated in numerous brain diseases, we studied miRNA expression and miRNA regulation of important signaling pathways during temporal lobe epileptogenesis in order to identify possible targets for epilepsy therapy. The temporal profile of miRNA expression was analyzed in thre...

journal_title：Neurobiology of disease

authors： Gorter JA,Iyer A,White I,Colzi A,van Vliet EA,Sisodiya S,Aronica E

更新日期：2014-02-01 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：: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：:Astrocytes are abundant neuron-supporting glial cells that harbor a powerful arsenal of neuroprotective antioxidative molecules and neurotrophic factors. Here we examined whether enrichment with healthy striatal astrocytes can provide neuroprotection against progressive dopaminergic neurodegeneration. Serotonin 1A (5-...

journal_title：Neurobiology of disease

authors： Miyazaki I,Asanuma M,Murakami S,Takeshima M,Torigoe N,Kitamura Y,Miyoshi K

更新日期：2013-11-01 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：:Although peroxisome biogenesis and β-oxidation disorders are well known for their neurodevelopmental defects, patients with these disorders are increasingly diagnosed with neurodegenerative pathologies. In order to investigate the cellular mechanisms of neurodegeneration in these patients, we developed a mouse model l...

journal_title：Neurobiology of disease

authors： Verheijden S,Bottelbergs A,Krysko O,Krysko DV,Beckers L,De Munter S,Van Veldhoven PP,Wyns S,Kulik W,Nave KA,Ramer MS,Carmeliet P,Kassmann CM,Baes M

更新日期：2013-10-01 00:00:00

abstract：:Mutations in LRRK2 are the most common genetic cause of Parkinson's disease (PD). The most prevalent LRRK2 mutation is the G2019S coding change, located in the kinase domain of this complex multi-domain protein. The majority of G2019S autopsy cases feature typical Lewy Body pathology with a clinical phenotype almost i...

journal_title：Neurobiology of disease

authors： Mamais A,Raja M,Manzoni C,Dihanich S,Lees A,Moore D,Lewis PA,Bandopadhyay R

更新日期：2013-10-01 00:00:00

abstract：:Excitotoxicity plays a central role in the neuronal damage during ischemic stroke. Although growing evidence suggests that activation of extrasynaptic NMDA receptors initiates neuronal death, no direct evidence demonstrated their activation during ischemia. Using rat hippocampal slices, we detected oxygen-glucose depr...

journal_title：Neurobiology of disease

authors： Dong QP,He JQ,Chai Z

更新日期：2013-10-01 00:00:00

abstract：:Mental illness is the leading cause of disability worldwide. We are only just beginning to reveal and comprehend the complex interaction that exists between the genetic makeup of an organism and the potential modifying effect of the environment in which it lives, and how this translates into mediating susceptibility t...

journal_title：Neurobiology of disease

authors： Renoir T,Pang TY,Hannan AJ

更新日期：2013-09-01 00:00:00

abstract：:Fragile X Syndrome (FXS) is a heritable form of mental retardation caused by a non-coding trinucleotide expansion of the FMR1 gene leading to loss of expression of this RNA binding protein. Mutations in this gene are strongly linked to enhanced Group I metabotropic glutamate receptor (mGluR) signaling. A recent report...

journal_title：Neurobiology of disease

authors： Straiker A,Min KT,Mackie K

更新日期：2013-08-01 00:00:00

abstract：:Spinocerebellar Ataxia Type 1 (SCA1) is an autosomal dominant late onset neurodegenerative disease caused by an expanded polyglutamine tract in ataxin-1. Here, we compared the protective effects of overexpressing ataxin-1-like using recombinant AAVs, or reducing expression of mutant ataxin-1 using virally delivered RN...

journal_title：Neurobiology of disease

authors： Keiser MS,Geoghegan JC,Boudreau RL,Lennox KA,Davidson BL

更新日期：2013-08-01 00:00:00

abstract：:GDAP1 is an outer mitochondrial membrane protein that acts as a regulator of mitochondrial dynamics. Mutations of the GDAP1 gene cause Charcot-Marie-Tooth (CMT) neuropathy. We show that GDAP1 interacts with the vesicle-organelle trafficking proteins RAB6B and caytaxin, which suggests that GDAP1 may participate in the ...

journal_title：Neurobiology of disease

authors： Pla-Martín D,Rueda CB,Estela A,Sánchez-Piris M,González-Sánchez P,Traba J,de la Fuente S,Scorrano L,Renau-Piqueras J,Alvarez J,Satrústegui J,Palau F

更新日期：2013-07-01 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：:Recent studies indicate that the Parkinson's disease-linked leucine-rich repeat kinase 2 (LRRK2) modulates cytoskeletal functions by regulating actin and tubulin dynamics, thereby affecting neurite outgrowth. By interactome analysis we demonstrate that the binding of LRRK2 to tubulins is significantly enhanced by phar...

journal_title：Neurobiology of disease

authors： Caesar M,Zach S,Carlson CB,Brockmann K,Gasser T,Gillardon F

更新日期：2013-06-01 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：:Symptoms in Parkinson's Disease (PD) have been linked to oscillatory activity within the basal ganglia. In humans, such activity has been detected mainly in the local field potentials (LFPs) recorded from electrode contacts used for deep brain stimulation. Although most studies have focused on activity within the subt...

journal_title：Neurobiology of disease

authors： Tsiokos C,Hu X,Pouratian N

更新日期：2013-06-01 00:00:00

abstract：:Toll-like receptors (TLRs) are mediators of the innate immune response to exogenous pathogens. They have also been implicated in sterile inflammation associated with systemic injury and non-infectious diseases via binding of endogenous ligands, possibly released by damaged cells. Emerging evidence indicates that some ...

journal_title：Neurobiology of disease

authors： David BT,Ratnayake A,Amarante MA,Reddy NP,Dong W,Sampath S,Heary RF,Elkabes S

更新日期：2013-06-01 00:00:00

abstract：:Spinal muscular atrophy (SMA), a genetic neurodegenerative disorder, is caused by mutations or deletions in the survival of motor neuron 1 (SMN1) gene that result in SMN deficiency. SMN deficiency impairs microtubule networks in Smn-deficient cells and in SMA-like motor neuron cultures. Microtubule defects can be rest...

journal_title：Neurobiology of disease

authors： Wen HL,Ting CH,Liu HC,Li H,Lin-Chao S

更新日期：2013-04-01 00:00:00

abstract：:Seizures occur in the basal ganglia (BG) of epileptic patients and in animal models of epilepsy, but there is relatively little known about how these events are gated and/or propagated through this structure. Here, we present and characterize a model of in vitro seizure-like events (SLEs) in the striatum by applying c...

journal_title：Neurobiology of disease

authors： Yu W,Calos M,Pilitsis J,Shin DS

更新日期：2013-04-01 00:00:00

abstract：:4-Aminopyridine (4AP, 50 μM) induces interictal- and ictal-like discharges in brain slices including parahippocampal areas such as the entorhinal cortex (EC) but the relation between these two types of epileptiform activity remains undifined. Here, by employing field potential recordings in rat EC slices during 4AP ap...

journal_title：Neurobiology of disease

authors： Avoli M,Panuccio G,Herrington R,D'Antuono M,de Guzman P,Lévesque M

更新日期：2013-04-01 00:00:00

abstract：:The optic nerve crush injury is a well-accepted model to study the mechanisms of axonal regeneration after trauma in the CNS. The infection of retinal ganglion cells (RGCs) with an adeno-associated virus serotype 2 - ciliary neurotrophic factor (AAV2.CNTF) was previously shown to stimulate axonal regeneration. However...

journal_title：Neurobiology of disease

authors： Pernet V,Joly S,Dalkara D,Jordi N,Schwarz O,Christ F,Schaffer DV,Flannery JG,Schwab ME

更新日期：2013-03-01 00:00:00

abstract：:We investigated two measures of neural integrity, T1-weighted volumetric measures and diffusion tensor imaging (DTI), and explored their combined potential to differentiate pre-diagnosis Huntington's disease (pre-HD) individuals from healthy controls. We applied quadratic discriminant analysis (QDA) to discriminate pr...

journal_title：Neurobiology of disease

authors： Georgiou-Karistianis N,Gray MA,Domínguez D JF,Dymowski AR,Bohanna I,Johnston LA,Churchyard A,Chua P,Stout JC,Egan GF

更新日期：2013-03-01 00:00:00

abstract：:It is widely accepted that the angiotensin AT2-receptor (AT2R) has neuroprotective features. In the present study we tested pharmacological AT2R-stimulation as a therapeutic approach in a model of spinal cord compression injury (SCI) in mice using the novel non-peptide AT2R-agonist, Compound 21 (C21). Complementary ex...

journal_title：Neurobiology of disease

authors： Namsolleck P,Boato F,Schwengel K,Paulis L,Matho KS,Geurts N,Thöne-Reineke C,Lucht K,Seidel K,Hallberg A,Dahlöf B,Unger T,Hendrix S,Steckelings UM

更新日期：2013-03-01 00:00:00

abstract：:Glutamate excitotoxicity is a consolidated hypothesis in neonatal brain injuries and tissue plasminogen activator (t-PA) participates in the processes through proteolytic and receptor mediated effects. In brain microvascular endothelial cell (nBMEC) cultures from neonates, t-PA content and release upon glutamate are h...

journal_title：Neurobiology of disease

authors： Henry VJ,Lecointre M,Laudenbach V,Ali C,Macrez R,Jullienne A,Berezowski V,Carmeliet P,Vivien D,Marret S,Gonzalez BJ,Leroux P

更新日期：2013-02-01 00:00:00

abstract：:Constitutive expression of C-C chemokine receptor (CCR) 5 has been detected in astrocytes, microglia and neurons, but its physiological roles in the central nervous system are obscure. The bidirectional interactions between neuron and glial cells through CCR5 and its ligands were thought to be crucial for maintaining ...

journal_title：Neurobiology of disease

authors： Choi DY,Lee MK,Hong JT

更新日期：2013-01-01 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：:Voltage-gated sodium channels (VGSCs) are essential for the generation and propagation of action potentials in electrically excitable cells. Dominant mutations in SCN1A, which encodes the Nav1.1 VGSC α-subunit, underlie several forms of epilepsy, including Dravet syndrome (DS) and genetic epilepsy with febrile seizure...

journal_title：Neurobiology of disease

authors： Dutton SB,Makinson CD,Papale LA,Shankar A,Balakrishnan B,Nakazawa K,Escayg A

更新日期：2013-01-01 00:00:00

abstract：:Matrix metalloproteinases (MMPs) are involved in tissue repair, cell death and morphogenesis. We investigated the role of the gelatinases MMP-2 and MMP-9 in the pathogenesis of neuronal death induced by prolonged seizures in the developing brain. Seven-day-old rats, MMP-9 knockout mice and transgenic rats overexpressi...

journal_title：Neurobiology of disease

authors： Hoehna Y,Uckermann O,Luksch H,Stefovska V,Marzahn J,Theil M,Gorkiewicz T,Gawlak M,Wilczynski GM,Kaczmarek L,Ikonomidou C

更新日期：2012-12-01 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：:Brain glucose supplies most of the carbon required for acetyl-coenzyme A (acetyl-CoA) generation (an important step for myelin synthesis) and for neurotransmitter production via further metabolism of acetyl-CoA in the tricarboxylic acid (TCA) cycle. However, it is not known whether reduced brain glucose transporter ty...

journal_title：Neurobiology of disease

authors： Marin-Valencia I,Good LB,Ma Q,Duarte J,Bottiglieri T,Sinton CM,Heilig CW,Pascual JM

更新日期：2012-10-01 00:00:00

abstract：:We recently reported evidence for disturbed synaptic versus extrasynaptic NMDAR transmission in the early pathogenesis of Huntington's disease (HD), a late-onset neurodegenerative disorder caused by CAG repeat expansion in the gene encoding huntingtin. Studies in glutamatergic cells indicate that synaptic NMDAR transm...

journal_title：Neurobiology of disease

authors： Milnerwood AJ,Kaufman AM,Sepers MD,Gladding CM,Zhang L,Wang L,Fan J,Coquinco A,Qiao JY,Lee H,Wang YT,Cynader M,Raymond LA

更新日期：2012-10-01 00:00:00

abstract：:Amyotrophic lateral sclerosis (ALS) is characterised by substantial loss of both upper and lower motor neuron function, with sensory and cognitive systems less affected. Though heritable forms of the disease have been described, the vast majority of cases are sporadic with poorly defined underlying pathogenic mechanis...

journal_title：Neurobiology of disease

authors： Peters OM,Millership S,Shelkovnikova TA,Soto I,Keeling L,Hann A,Marsh-Armstrong N,Buchman VL,Ninkina N

更新日期：2012-10-01 00:00:00

abstract：:Huntington's disease (HD) is a genetic neurodegenerative disease characterized by movement disorders, cognitive decline and neuropsychiatric symptoms. HD is caused by expanded CAG tract within the coding region of Huntingtin protein. Despite major insights into the molecular mechanisms leading to HD, no effective cure...

journal_title：Neurobiology of disease

authors： Dupont P,Besson MT,Devaux J,Liévens JC

更新日期：2012-08-01 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：PURPOSE:Generalized spike and wave discharges (SWD) are generated within the cortico-thalamo-cortical system. However the exact interactions between cortex and different thalamic nuclei needed for the generation and maintenance of SWD are still to be elucidated. This study aims to shed more light on these interactions ...

journal_title：Neurobiology of disease

authors： Lüttjohann A,van Luijtelaar G

更新日期：2012-07-01 00:00:00