Increased slow oscillatory activity in substantia nigra pars reticulata triggers abnormal involuntary movements in the 6-OHDA-lesioned rat in the presence of excessive extracellular striatal dopamine.

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：:Glutamate-induced delayed calcium dysregulation (DCD) is a causal factor leading to neuronal death. The mechanism of DCD is not clear but Ca2+ influx via N-methyl-d-aspartate receptors (NMDAR) and/or the reverse plasmalemmal Na+/Ca2+ exchanger (NCXrev) could be involved in DCD. However, the extent to which NMDAR and N...

journal_title：Neurobiology of disease

authors： Brittain MK,Brustovetsky T,Sheets PL,Brittain JM,Khanna R,Cummins TR,Brustovetsky N

更新日期：2012-04-01 00:00:00

abstract：:In a transgenic mouse model of the neurodegenerative disorder Huntington's disease (HD), age-dependent neurologic defects are accompanied by progressive alterations in glucose tolerance that culminate in the development of diabetes mellitus and insulin deficiency. Pancreatic islets from HD transgenic mice express redu...

journal_title：Neurobiology of disease

authors： Andreassen OA,Dedeoglu A,Stanojevic V,Hughes DB,Browne SE,Leech CA,Ferrante RJ,Habener JF,Beal MF,Thomas MK

更新日期：2002-12-01 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：:Charcot-Marie-Tooth disease (CMT) is a genetic disorder that can be caused by aberrations in >80 genes. CMT has heterogeneous modes of inheritance, including autosomal dominant, autosomal recessive, X-linked dominant, and X-linked recessive. Over 95% of cases are dominantly inherited. In this study, we investigated wh...

journal_title：Neurobiology of disease

authors： Lee JS,Chang EH,Koo OJ,Jwa DH,Mo WM,Kwak G,Moon HW,Park HT,Hong YB,Choi BO

更新日期：2017-04-01 00:00:00

abstract：:Early exposure to general anesthesia (GA) causes developmental neuroapoptosis in the mammalian brain and long-term cognitive impairment. Recent evidence suggests that GA also causes functional and morphological impairment of the immature neuronal mitochondria. Injured mitochondria could be a significant source of reac...

journal_title：Neurobiology of disease

authors： Boscolo A,Starr JA,Sanchez V,Lunardi N,DiGruccio MR,Ori C,Erisir A,Trimmer P,Bennett J,Jevtovic-Todorovic V

更新日期：2012-03-01 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：:We investigated the effects of engrafted human adult olfactory neuroepithelial neurosphere forming cells (NSFCs) on regeneration and reinnervation of rubrospinal tract (RST) axons and locomotor recovery following partial cervical hemisection that completely ablated the RST. Weekly behavioral testing showed greater fun...

journal_title：Neurobiology of disease

authors： Xiao M,Klueber KM,Guo Z,Lu C,Wang H,Roisen FJ

更新日期：2007-05-01 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：:Loss of function mutations in the SCN1A gene, which encodes the voltage-gated sodium channel Nav1.1, have been described in the majority of Dravet syndrome patients presenting with epileptic seizures, hyperactivity, autistic traits, and cognitive decline. We previously reported predominant Nav1.1 expression in parvalb...

journal_title：Neurobiology of disease

authors： Tatsukawa T,Ogiwara I,Mazaki E,Shimohata A,Yamakawa K

更新日期：2018-04-01 00:00:00

abstract：:Both fragile X mental retardation protein (FMRP) and brain-derived neurotrophic factor (BDNF) are implicated in the maturation of neurons and in the higher cognitive functions. We have investigated whether FMRP and BDNF are reciprocally regulated in neurons. Exposure of cultured hippocampal neurons to BDNF, but not to...

journal_title：Neurobiology of disease

authors： Castrén M,Lampinen KE,Miettinen R,Koponen E,Sipola I,Bakker CE,Oostra BA,Castrén E

更新日期：2002-10-01 00:00:00

abstract：:Decreased release probability (Pr) and increased failure rate for monosynaptic inhibitory postsynaptic currents (IPSCs) indicate abnormalities in presynaptic inhibitory terminals on pyramidal (Pyr) neurons of the undercut (UC) model of posttraumatic epileptogenesis. These indices of inhibition are normalized in high [...

journal_title：Neurobiology of disease

authors： Faria LC,Parada I,Prince DA

更新日期：2012-02-01 00:00:00

abstract：:Infantile neuronal ceroid lipofuscinosis (INCL) is a severe neurodegenerative disorder of children, characterized by selective death of neocortical neurons. To understand early disease mechanisms in INCL, we have studied Ppt1(Deltaex4) knock-out mouse neurons in culture and acute brain slices. Global transcript profil...

journal_title：Neurobiology of disease

authors： Ahtiainen L,Kolikova J,Mutka AL,Luiro K,Gentile M,Ikonen E,Khiroug L,Jalanko A,Kopra O

更新日期：2007-10-01 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：: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：:Long-term consequences of status epilepticus (SE) occur in a significant proportion of those who survive the acute episode. We developed an in vivo model of acute focal neocortical SE (FSE) to study long-term effects on local cortical structure and function and potential strategies to mitigate adverse consequences of ...

journal_title：Neurobiology of disease

authors： Perez-Ramirez MB,Gu F,Prince DA

更新日期：2020-08-01 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：:Amyloid-β is a peptide released by synapses in physiological conditions and its pathological accumulation in brain structures necessary for memory processing represents a key toxic hallmark underlying Alzheimer's disease. The oligomeric form of Amyloid-β (Aβο) is now believed to represent the main Amyloid-β species af...

journal_title：Neurobiology of disease

authors： Kootar S,Frandemiche ML,Dhib G,Mouska X,Lorivel T,Poupon-Silvestre G,Hunt H,Tronche F,Bethus I,Barik J,Marie H

更新日期：2018-10-01 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：:We have used a quantitative in situ hybridization method with human ribonucleotide probes to examine the regional and cellular distribution of N-methyl-D-aspartate receptor (NMDAR) subunit mRNAs in the human cerebellum. Purkinje cells showed very dense labeling for NMDAR1 mRNA, dense labeling for NMDAR2A mRNA, and mod...

journal_title：Neurobiology of disease

authors： Scherzer CR,Landwehrmeyer GB,Kerner JA,Standaert DG,Hollingsworth ZR,Daggett LP,Veliçelebi G,Penney JB Jr,Young AB

更新日期：1997-01-01 00:00:00

abstract：:Alzheimer disease (AD) is the most prevalent form of dementia. Loss of hippocampal synapses is the first neurodegenerative event in AD. Synaptic loss has been associated with the accumulation in the brain parenchyma of soluble oligomeric forms of amyloid β peptide (Aβ1-42wt). Clinical observations have shown that a mu...

journal_title：Neurobiology of disease

authors： Cimini S,Sclip A,Mancini S,Colombo L,Messa M,Cagnotto A,Di Fede G,Tagliavini F,Salmona M,Borsello T

更新日期：2016-05-01 00:00:00

abstract：:Cerebral autosomal dominant arteriopathy with subcortical infarct and leukoencephalopathy (CADASIL) is a cerebral small vascular disease caused by NOTCH3 gene mutation in vascular smooth muscle cells (VSMCs), leading to ischemic stroke and vascular dementia. To date, the pathogenesis of CADASIL remains poorly understo...

journal_title：Neurobiology of disease

authors： Ping S,Qiu X,Kyle M,Hughes K,Longo J,Zhao LR

更新日期：2019-12-01 00:00:00

abstract：UNLABELLED:Mitochondrial dysfunction is involved in the pathogenesis of motor neuron degeneration in the G93A mutant transgenic (tgmSOD1) animal model of ALS. However, it is unknown whether mitochondriopathy is a primary or secondary event. We isolated brain (BM) and spinal cord (SCM) mitochondria from 2 month old pres...

journal_title：Neurobiology of disease

authors： Panov A,Kubalik N,Zinchenko N,Hemendinger R,Dikalov S,Bonkovsky HL

更新日期：2011-10-01 00:00:00

abstract：:Premature stop codons in CACNA1A, which encodes the alpha(1A) subunit of neuronal P/Q-type (Ca(V)2.1) Ca(2+) channels, cause episodic ataxia type 2 (EA2). CACNA1A undergoes extensive alternative splicing, which contributes to the pharmacological and kinetic heterogeneity of Ca(V)2.1-mediated Ca(2+) currents. We identi...

journal_title：Neurobiology of disease

authors： Graves TD,Imbrici P,Kors EE,Terwindt GM,Eunson LH,Frants RR,Haan J,Ferrari MD,Goadsby PJ,Hanna MG,van den Maagdenberg AM,Kullmann DM

更新日期：2008-10-01 00:00:00

abstract：:In a comprehensive proteomics study aiming at the identification of proteins associated with amyloid-beta (Abeta)-mediated toxicity in cultured cortical neurons, we have identified Thimet oligopeptidase (THOP1). Functional modulation of THOP1 levels in primary cortical neurons demonstrated that its overexpression was ...

journal_title：Neurobiology of disease

authors： Pollio G,Hoozemans JJ,Andersen CA,Roncarati R,Rosi MC,van Haastert ES,Seredenina T,Diamanti D,Gotta S,Fiorentini A,Magnoni L,Raggiaschi R,Rozemuller AJ,Casamenti F,Caricasole A,Terstappen GC

更新日期：2008-07-01 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：:The TATA-box binding protein associated factor 1 (TAF1) is part of the TFIID complex that plays a key role during the initiation of transcription. Variants of TAF1 are associated with neurodevelopmental disorders. Previously, we found that CRISPR/Cas9 based editing of the TAF1 gene disrupts the morphology of the cereb...

journal_title：Neurobiology of disease

authors： Dhanalakshmi C,Janakiraman U,Moutal A,Fukunaga K,Khanna R,Nelson MA

更新日期：2021-02-01 00:00:00

abstract：:In this review, we describe insights into beta-amyloid (Abeta) production using aged dogs as a model of human brain aging. The advantage of using dogs is that they naturally accumulate Abeta neuropathology with age. In parallel, dogs also develop age-associated learning and memory impairments. Thus, dogs can complemen...

journal_title：Neurobiology of disease

authors： Head E,Torp R

更新日期：2002-02-01 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：: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