abstract：:Clinical and experimental evidence point to a possible role of cerebrovascular dysfunction in Alzheimer's disease (AD). The 5xFAD mouse model of AD expresses human amyloid precursor protein and presenilin genes with mutations found in AD patients. It remains unknown whether amyloid deposition driven by these mutations...

journal_title：Neurobiology of disease

authors： Giannoni P,Arango-Lievano M,Neves ID,Rousset MC,Baranger K,Rivera S,Jeanneteau F,Claeysen S,Marchi N

更新日期：2016-04-01 00:00:00

abstract：:Fragile X syndrome (FXS) patients do not make the fragile X mental retardation protein (FMRP). The absence of FMRP causes dysregulated translation, abnormal synaptic plasticity and the most common form of inherited intellectual disability. But FMRP loss has minimal effects on memory itself, making it difficult to unde...

journal_title：Neurobiology of disease

authors： Radwan B,Dvorak D,Fenton AA

更新日期：2016-04-01 00:00:00

abstract：:Aberrant brain iron deposition is observed in both common and rare neurodegenerative disorders, including those categorized as Neurodegeneration with Brain Iron Accumulation (NBIA), which are characterized by focal iron accumulation in the basal ganglia. Two NBIA genes are directly involved in iron metabolism, but whe...

journal_title：Neurobiology of disease

authors： Bettencourt C,Forabosco P,Wiethoff S,Heidari M,Johnstone DM,Botía JA,Collingwood JF,Hardy J,UK Brain Expression Consortium (UKBEC).,Milward EA,Ryten M,Houlden H

更新日期：2016-03-01 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：:Huntington's disease (HD) is an inherited neurodegenerative disorder characterized by dyskinesia, cognitive impairment and emotional disturbances, presenting progressive neurodegeneration in the striatum and intracellular mutant Huntingtin (mHTT) aggregates in various areas of the brain. Recombinant Adeno Associated V...

journal_title：Neurobiology of disease

authors： Ceccarelli I,Fiengo P,Remelli R,Miragliotta V,Rossini L,Biotti I,Cappelli A,Petricca L,La Rosa S,Caricasole A,Pollio G,Scali C

更新日期：2016-02-01 00:00:00

abstract：:This paper presents a novel model of tremor in Parkinson's disease (PD) based on extensive literature review as well as novel results stemming from functional stereotactic neurosurgery for the alleviation of tremor in PD. Specifically, evidence that suggests the basal ganglia induces PD tremor via excessive inhibitory...

journal_title：Neurobiology of disease

authors： Duval C,Daneault JF,Hutchison WD,Sadikot AF

更新日期：2016-01-01 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：:Autophagy and redox biochemistry are two major sub disciplines of cell biology which are both coming to be appreciated for their paramount importance in the etiology of neurodegenerative diseases including Alzheimer's disease (AD). Thus far, however, there has been relatively little exploration of the interface betwee...

journal_title：Neurobiology of disease

authors： Hensley K,Harris-White ME

更新日期：2015-12-01 00:00:00

abstract：:Somatosensation plays a critical role in the dexterous manipulation of objects, in emotional communication, and in the embodiment of our limbs. For upper-limb neuroprostheses to be adopted by prospective users, prosthetic limbs will thus need to provide sensory information about the position of the limb in space and a...

journal_title：Neurobiology of disease

authors： Tabot GA,Kim SS,Winberry JE,Bensmaia SJ

更新日期：2015-11-01 00:00:00

abstract：:Bradykinesia is the most important feature contributing to motor difficulties in Parkinson's disease (PD). However, the pathophysiology underlying bradykinesia is not fully understood. One important aspect is that PD patients have difficulty in performing learned motor skills automatically, but this problem has been g...

journal_title：Neurobiology of disease

authors： Wu T,Hallett M,Chan P

更新日期：2015-10-01 00:00:00

abstract：:Cannabis is an increasingly popular and controversial drug used worldwide. Cannabis use often begins during adolescence, a highly susceptible period for environmental stimuli to alter functional and structural organization of the developing brain. Given that adolescence is a critical time for the emergence of mental i...

journal_title：Neurobiology of disease

authors： Ballinger MD,Saito A,Abazyan B,Taniguchi Y,Huang CH,Ito K,Zhu X,Segal H,Jaaro-Peled H,Sawa A,Mackie K,Pletnikov MV,Kamiya A

更新日期：2015-10-01 00:00:00

abstract：:The protracted and age-dependent degeneration of dopamine (DA)-producing neurons of the Substantia nigra pars compacta (SNc) and ventral tegmental area (VTA) in the mammalian midbrain is a hallmark of human Parkinson's Disease (PD) and of certain genetic mouse models of PD, such as mice heterozygous for the homeodomai...

journal_title：Neurobiology of disease

authors： Zhang J,Götz S,Vogt Weisenhorn DM,Simeone A,Wurst W,Prakash N

更新日期：2015-10-01 00:00:00

abstract：:Studies have showed that prenatal cocaine exposure (PCOC) can impair cognitive function and social behavior of the offspring; however, the mechanism underlying such effect is poorly understood. Insulin-like growth factor II (Igf-II), an imprinted gene, has a critical role in memory consolidation and enhancement. We hy...

journal_title：Neurobiology of disease

authors： Zhao Q,Hou J,Chen B,Shao X,Zhu R,Bu Q,Gu H,Li Y,Zhang B,Du C,Fu D,Kong J,Luo L,Long H,Li H,Deng Y,Zhao Y,Cen X

更新日期：2015-10-01 00:00:00

abstract：:Low brain expression of the spermidine/spermine N-1 acetyltransferase (SAT1) gene, the rate-limiting enzyme involved in catabolism of polyamines that mediate the polyamine stress response (PSR), has been reported in depressed suicides. However, it is unknown whether this effect is associated with depression or with su...

journal_title：Neurobiology of disease

authors： Pantazatos SP,Andrews SJ,Dunning-Broadbent J,Pang J,Huang YY,Arango V,Nagy PL,John Mann J

更新日期：2015-07-01 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：:Substantial interest persists for developing neurotrophic factors to treat neurodegenerative diseases. At the same time, significant progress has been made in implementing gene therapy as a means to provide long-term expression of bioactive neurotrophic factors to targeted sites in the brain. Nonetheless, to date, no ...

journal_title：Neurobiology of disease

authors： Bartus RT,Kordower JH,Johnson EM Jr,Brown L,Kruegel BR,Chu Y,Baumann TL,Lang AE,Olanow CW,Herzog CD

更新日期：2015-06-01 00:00:00

abstract：:Mutations in the LRRK2 gene represent the most common genetic cause of late onset Parkinson's disease. The physiological and pathological roles of LRRK2 are yet to be fully determined but evidence points towards LRRK2 mutations causing a gain in kinase function, impacting on neuronal maintenance, vesicular dynamics an...

journal_title：Neurobiology of disease

authors： Yue M,Hinkle KM,Davies P,Trushina E,Fiesel FC,Christenson TA,Schroeder AS,Zhang L,Bowles E,Behrouz B,Lincoln SJ,Beevers JE,Milnerwood AJ,Kurti A,McLean PJ,Fryer JD,Springer W,Dickson DW,Farrer MJ,Melrose HL

更新日期：2015-06-01 00:00:00

abstract：:The unfolded protein response (UPR) monitors the folding environment within the endoplasmic reticulum (ER). Accumulation of misfolded proteins within the ER activates the UPR resulting in the execution of adaptive or non-adaptive signaling pathways. α-Synuclein (α-syn) whose accumulation and aggregation define the pat...

journal_title：Neurobiology of disease

authors： Credle JJ,Forcelli PA,Delannoy M,Oaks AW,Permaul E,Berry DL,Duka V,Wills J,Sidhu A

更新日期：2015-04-01 00:00:00

abstract：:Amyotrophic lateral sclerosis (ALS) is a rapidly progressive and ultimately fatal neurodegenerative disease, caused by the loss of motor neurons in the brain and spinal cord. Although 10% of ALS cases are familial (FALS), the majority are sporadic (SALS) and probably associated to a multifactorial etiology. Currently ...

journal_title：Neurobiology of disease

authors： Aronica E,Baas F,Iyer A,ten Asbroek AL,Morello G,Cavallaro S

更新日期：2015-02-01 00:00:00

abstract：:We have previously shown that increases in blood-brain barrier permeability represent an important component of ischemia-reperfusion related brain injury in the fetus. Pro-inflammatory cytokines could contribute to these abnormalities in blood-brain barrier function. We have generated pharmacological quantities of mou...

journal_title：Neurobiology of disease

authors： Chen X,Sadowska GB,Zhang J,Kim JE,Cummings EE,Bodge CA,Lim YP,Makeyev O,Besio WG,Gaitanis J,Threlkeld SW,Banks WA,Stonestreet BS

更新日期：2015-01-01 00:00:00

abstract：:Inflammation is a growing area of research in neurodegeneration. In Huntington's disease (HD), a fatal inherited neurodegenerative disease caused by a CAG-repeat expansion in the gene encoding huntingtin, patients have increased plasma levels of inflammatory cytokines and circulating monocytes that are hyper-responsiv...

journal_title：Neurobiology of disease

authors： Träger U,Andre R,Magnusson-Lind A,Miller JR,Connolly C,Weiss A,Grueninger S,Silajdžić E,Smith DL,Leavitt BR,Bates GP,Björkqvist M,Tabrizi SJ

更新日期：2015-01-01 00:00:00

abstract：:Extracellular matrix molecule chondroitin sulfate proteoglycans (CSPGs) are highly upregulated in scar tissues and form a potent chemical barrier for CNS axon regeneration. Recent studies support that the receptor protein tyrosine phosphatase σ (PTPσ) and its subfamily member leukocyte common antigen related phosphata...

journal_title：Neurobiology of disease

authors： Xu B,Park D,Ohtake Y,Li H,Hayat U,Liu J,Selzer ME,Longo FM,Li S

更新日期：2015-01-01 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：:Mutations in vacuolar protein sorting 35 (VPS35) have been linked to familial Parkinson's disease (PD). VPS35, a component of the retromer, mediates the retrograde transport of cargo from the endosome to the trans-Golgi network. Here we showed that retromer depletion increases the lysosomal turnover of the mannose 6-p...

journal_title：Neurobiology of disease

authors： Miura E,Hasegawa T,Konno M,Suzuki M,Sugeno N,Fujikake N,Geisler S,Tabuchi M,Oshima R,Kikuchi A,Baba T,Wada K,Nagai Y,Takeda A,Aoki M

更新日期：2014-11-01 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：:Parkinson's disease (PD), characterized by the loss of dopaminergic nigrostriatal projections, is a debilitating neurodegenerative disease which produces bradykinesia, rigidity, tremor and postural instability. The dopamine precursor levodopa (L-Dopa) is the most effective treatment for the amelioration of PD signs an...

journal_title：Neurobiology of disease

authors： Prescott IA,Liu LD,Dostrovsky JO,Hodaie M,Lozano AM,Hutchison WD

更新日期：2014-11-01 00:00:00

abstract：:Neuroinflammatory changes play a pivotal role in the progression of Parkinson's disease (PD) pathogenesis. Recent findings have suggested that activated microglia may polarize similarly to peripheral macrophages in the central nervous system (CNS), assuming a pro-inflammatory M1 phenotype or the alternative anti-infla...

journal_title：Neurobiology of disease

authors： Pisanu A,Lecca D,Mulas G,Wardas J,Simbula G,Spiga S,Carta AR

更新日期：2014-11-01 00:00:00

abstract：:Repeated head injuries are a major public health concern both for athletes, and members of the police and armed forces. There is ample experimental and clinical evidence that there is a period of enhanced vulnerability to subsequent injury following head trauma. Injuries that occur close together in time produce great...

journal_title：Neurobiology of disease

authors： Weil ZM,Gaier KR,Karelina K

更新日期：2014-10-01 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：:G-protein coupled receptor 3 (GPR3), GPR6, and GPR12 belong to a family of constitutively active Gs-coupled receptors that activate 3'-5'-cyclic adenosine monophosphate (cAMP) and are highly expressed in the brain. Among these receptors, the endogenous expression of GPR3 in cerebellar granule neurons (CGNs) is increas...

journal_title：Neurobiology of disease

authors： Tanaka S,Miyagi T,Dohi E,Seki T,Hide I,Sotomaru Y,Saeki Y,Antonio Chiocca E,Matsumoto M,Sakai N

更新日期：2014-08-01 00:00:00

abstract：:Twelve- to sixteen-month-old (G2019S) LRRK2 transgenic mice prepared by us displayed progressive neuronal death of substantia nigra pars compacta (SNpc) dopaminergic cells. In the present study, we hypothesized that prior to a late-phase death of SNpc dopaminergic neurons, (G2019S) LRRK2 also causes an early-phase neu...

journal_title：Neurobiology of disease

authors： Chou JS,Chen CY,Chen YL,Weng YH,Yeh TH,Lu CS,Chang YM,Wang HL

更新日期：2014-08-01 00:00:00

abstract：:All tauopathies result in various forms of cognitive decline and neuronal loss. Although in some diseases, tau mutations appear to cause neurodegeneration, the toxic "form" of tau remains elusive. Tau is the major protein found within neurofibrillary tangles (NFTs) and therefore it seemed rational to assume that aggre...

journal_title：Neurobiology of disease

authors： Ward SM,Himmelstein DS,Ren Y,Fu Y,Yu XW,Roberts K,Binder LI,Sahara N

更新日期：2014-07-01 00:00:00

abstract：:Optic neuritis is a frequent and early symptom of multiple sclerosis (MS). Conventional magnetic resonance (MR) techniques provide means to assess multiple MS-related pathologies, including axonal injury, demyelination, and inflammation. A method to directly and non-invasively probe white-matter function could further...

journal_title：Neurobiology of disease

authors： Lin TH,Spees WM,Chiang CW,Trinkaus K,Cross AH,Song SK

更新日期：2014-07-01 00:00:00

abstract：:Gephyrin is a postsynaptic scaffolding protein, essential for the clustering of glycine and γ-aminobutyric acid type-A receptors (GABAARs) at inhibitory synapses. An impairment of GABAergic synaptic inhibition represents a key pathway of epileptogenesis. Recently, exonic microdeletions in the gephyrin (GPHN) gene have...

journal_title：Neurobiology of disease

authors： Dejanovic B,Lal D,Catarino CB,Arjune S,Belaidi AA,Trucks H,Vollmar C,Surges R,Kunz WS,Motameny S,Altmüller J,Köhler A,Neubauer BA,Epicure Consortium,Nürnberg P,Noachtar S,Schwarz G,Sander T

更新日期：2014-07-01 00:00:00

abstract：:Charcot-Marie-Tooth disease type 2B (CMT2B) is an inherited axonal peripheral neuropathy. It is characterised by prominent sensory loss, often complicated by severe ulcero-mutilations of toes or feet, and variable motor involvement. Missense mutations in RAB7A, the gene encoding the small GTPase Rab7, cause CMT2B and ...

journal_title：Neurobiology of disease

authors： Janssens K,Goethals S,Atkinson D,Ermanoska B,Fransen E,Jordanova A,Auer-Grumbach M,Asselbergh B,Timmerman V

更新日期：2014-05-01 00:00:00

abstract：:Type 2 diabetes (T2D) is a major risk factor for late-onset Alzheimer's disease (AD). A variety of metabolic changes related to T2D (e.g. hyperinsulinemia, hyperglycemia, and elevated branched-chain amino acids) have been proposed as mechanistic links, but the basis for this association remains unknown. Retromer-depen...

journal_title：Neurobiology of disease

authors： Morabito MV,Berman DE,Schneider RT,Zhang Y,Leibel RL,Small SA

更新日期：2014-05-01 00:00:00

abstract：OBJECTIVE:To investigate structural connectivity and the relationship between axonal microstructure and clinical, cognitive, and motor functions in premanifest (pre-HD) and symptomatic (symp-HD) Huntington's disease. METHOD:Diffusion tensor imaging (DTI) data were acquired from 35 pre-HD, 36 symp-HD, and 35 controls. ...

journal_title：Neurobiology of disease

authors： Poudel GR,Stout JC,Domínguez D JF,Salmon L,Churchyard A,Chua P,Georgiou-Karistianis N,Egan GF

更新日期：2014-05-01 00:00:00

abstract：:The spreading of pathology through neuronal pathways is likely to be the cause of the progressive cognitive loss observed in Alzheimer's disease (AD) and other neurodegenerative diseases. We have recently shown the propagation of AD pathology via cell-to-cell transfer of oligomeric amyloid beta (Aβ) residues 1-42 (oAβ...

journal_title：Neurobiology of disease

authors： Domert J,Rao SB,Agholme L,Brorsson AC,Marcusson J,Hallbeck M,Nath S

更新日期：2014-05-01 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：:The most appropriate time for the initiation of dopaminergic symptomatic therapy in Parkinson's disease remains debatable. It has been suggested that early correction of basal ganglia pathophysiological abnormalities may have long-term beneficial effects. To test this hypothesis, we investigated the early and delayed ...

journal_title：Neurobiology of disease

authors： Marin C,Bonastre M,Mengod G,Cortés R,Giralt A,Obeso JA,Schapira AH

更新日期：2014-04-01 00:00:00