No differences of butyrylcholinesterase protein activity and allele frequency in Lewy body diseases.

abstract：:A wide spectrum of focal, regional, or diffuse structural brain abnormalities, collectively known as malformations of cortical development (MCDs), frequently manifest with intellectual disability (ID), epilepsy, and/or autistic spectrum disorder (ASD). As the acronym suggests, MCDs are perturbations of the normal arch...

journal_title：Neurobiology of disease

authors： Stouffer MA,Golden JA,Francis F

更新日期：2016-08-01 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：:Neuropeptide Y (NPY) is an important 36 amino acid peptide that is abundantly expressed in the mammalian CNS and is known to be an endogenous modulator of seizure activity, including in rat models of Genetic Generalised Epilepsy (GGE) with absence seizures. Studies have shown that viral-mediated "gene therapy" with ov...

journal_title：Neurobiology of disease

authors： Powell KL,Fitzgerald X,Shallue C,Jovanovska V,Klugmann M,Von Jonquieres G,O'Brien TJ,Morris MJ

更新日期：2018-05-01 00:00:00

abstract：:Familial amyotrophic lateral sclerosis (FALS) has been modeled in transgenic mice by introducing mutated versions of human genomic DNA encompassing the entire gene for Cu,Zn superoxide dismutase (SOD1). In this setting, the transgene is expressed throughout the body and results in mice that faithfully recapitulate man...

journal_title：Neurobiology of disease

authors： Wang J,Xu G,Slunt HH,Gonzales V,Coonfield M,Fromholt D,Copeland NG,Jenkins NA,Borchelt DR

更新日期：2005-12-01 00:00:00

abstract：:Huntington's disease (HD) and Dentatorubral and pallidoluysian atrophy (DRPLA) are autosomal dominant, neurodegenerative disorders caused by the expansion of polyglutamine tracts in their respective proteins, huntingtin and atrophin-1. We have previously generated mouse models of these disorders, using transgenes expr...

journal_title：Neurobiology of disease

authors： Schilling G,Jinnah HA,Gonzales V,Coonfield ML,Kim Y,Wood JD,Price DL,Li XJ,Jenkins N,Copeland N,Moran T,Ross CA,Borchelt DR

更新日期：2001-06-01 00:00:00

abstract：:Vascular cognitive impairment is a major cause of dementia caused by chronic hypoxia, producing progressive damage to white matter (WM) secondary to blood-brain barrier (BBB) opening and vascular dysfunction. Tight junction proteins (TJPs), which maintain BBB integrity, are lost in acute ischemia. Although angiogenesi...

journal_title：Neurobiology of disease

authors： Yang Y,Kimura-Ohba S,Thompson JF,Salayandia VM,Cossé M,Raz L,Jalal FY,Rosenberg GA

更新日期：2018-06-01 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：: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：: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：: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：:Prion diseases are disorders of protein conformation that produce neurodegeneration in humans and animals. Studies of transgenic (Tg) mice indicate that a factor designated protein X is involved in the conversion of the normal cellular prion protein (PrPC) into the scrapie isoform (PrPSc); protein X appears to interac...

journal_title：Neurobiology of disease

authors： Yehiely F,Bamborough P,Da Costa M,Perry BJ,Thinakaran G,Cohen FE,Carlson GA,Prusiner SB

更新日期：1997-01-01 00:00:00

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：:Patients with Krabbe disease, a genetic demyelinating syndrome caused by deficiency of galactosyl-ceramidase and the resulting accumulation of galactosyl-sphingolipids, develop signs of a dying-back axonopathy compounded by a deficiency of large-caliber axons. Here, we show that axonal caliber in Twitcher mice, an ani...

journal_title：Neurobiology of disease

authors： Cantuti-Castelvetri L,Zhu H,Givogri MI,Chidavaenzi RL,Lopez-Rosas A,Bongarzone ER

更新日期：2012-05-01 00:00:00

abstract：:Possible roles of the complement (C) system in the normal and injured brain were explored with inbred mice that carried a frameshift mutation in the C5 gene. A congenic pair was used: the C5-sufficient (C5+) B10.D2/nSnJ strain with the functional allele (Hc1) from the C57BL/10J donor strain was compared with the C5-de...

journal_title：Neurobiology of disease

authors： Pasinetti GM,Tocco G,Sakhi S,Musleh WD,DeSimoni MG,Mascarucci P,Schreiber S,Baudry M,Finch CE

更新日期：1996-01-01 00:00:00

abstract：:Human genetics provides unbiased insights into the causes of human disease, which can be used to create a foundation for effective ways to more accurately diagnose patients, stratify patients for more successful clinical trials, discover and develop new therapies, and ultimately help patients choose the safest and mos...

journal_title：Neurobiology of disease

authors： Finkbeiner S

更新日期：2020-12-01 00:00:00

abstract：:As researchers across the globe have focused their attention on understanding SARS-CoV-2, the picture that is emerging is that of a virus that has serious effects on the vasculature in multiple organ systems including the cerebral vasculature. Observed effects on the central nervous system include neurological symptom...

journal_title：Neurobiology of disease

authors： Buzhdygan TP,DeOre BJ,Baldwin-Leclair A,Bullock TA,McGary HM,Khan JA,Razmpour R,Hale JF,Galie PA,Potula R,Andrews AM,Ramirez SH

更新日期：2020-12-01 00:00:00

abstract：:We have studied the neurotoxicity of amyloid-beta (Abeta) after a single unilateral intravitreal injection. Within the retina apoptotic cells were seen throughout the photoreceptor layer and the inner nuclear layer but not in the ganglion cell layer at 48 h after injection of Abeta(1-42) compared to vehicle control an...

journal_title：Neurobiology of disease

authors： Walsh DT,Montero RM,Bresciani LG,Jen AY,Leclercq PD,Saunders D,EL-Amir AN,Gbadamoshi L,Gentleman SM,Jen LS

更新日期：2002-06-01 00:00:00

abstract：:Microtubule Associated Protein Tau (MAPT) forms proteopathic aggregates in several diseases. The G273R tau mutation, located in the first repeat region, was found by exome sequencing in a patient who presented with dementia and parkinsonism. We herein return to pathological examination which demonstrated tau immunorea...

journal_title：Neurobiology of disease

authors： Sandberg A,Ling H,Gearing M,Dombroski B,Cantwell L,R'Bibo L,Levey A,Schellenberg GD,Hardy J,Wood N,Fernius J,Nyström S,Svensson S,Thor S,Hammarström P,Revesz T,Mok KY

更新日期：2020-12-01 00:00:00

abstract：:There is evidence that cognitive decline in Alzheimer's disease (AD) results from deficiencies in synaptic communication (e.g., loss of mushroom-shaped 'memory spines') and neurodegenerative processes. This might be treated with sigma-1 receptor (S1R) agonists, which are broadly neuroprotective and modulate synaptic p...

journal_title：Neurobiology of disease

authors： Ryskamp D,Wu L,Wu J,Kim D,Rammes G,Geva M,Hayden M,Bezprozvanny I

更新日期：2019-04-01 00:00:00

abstract：:The repertoire of biochemicals (or small molecules) present in cells, tissue, and body fluids is known as the metabolome. Today, clinicians utilize only a very small part of the information contained in the metabolome, as revealed by the quantification of a limited set of analytes to gain information on human health. ...

journal_title：Neurobiology of disease

authors： Quinones MP,Kaddurah-Daouk R

更新日期：2009-08-01 00:00:00

abstract：:Transglutaminase 2 (TG2) is a multifunctional protein that modulates cell survival and death pathways. It is upregulated in numerous ischemic models, and protects primary neurons from oxygen and glucose deprivation. TG2 binds to the hypoxia inducible factor (HIF) 1beta and decreases the upregulation of hypoxic-induced...

journal_title：Neurobiology of disease

authors： Filiano AJ,Tucholski J,Dolan PJ,Colak G,Johnson GV

更新日期：2010-09-01 00:00:00

abstract：:The amyloid precursor protein (APP) metabolism is central to pathogenesis of Alzheimer's disease (AD). Parenchymal amyloid deposits, a neuropathological hallmark of AD, are composed of amyloid-beta peptides (Abeta). Abeta derives from the amyloid precursor protein (APP) by sequential cleavages by beta- and gamma-secre...

journal_title：Neurobiology of disease

authors： Vingtdeux V,Hamdane M,Bégard S,Loyens A,Delacourte A,Beauvillain JC,Buée L,Marambaud P,Sergeant N

更新日期：2007-03-01 00:00:00

abstract：:The neuroprotective effect of L-kynurenine sulfate (KYN), a precursor of kynurenic acid (KYNA, a selective N-methyl-D-aspartate receptor antagonist), was studied. KYN (300 mg/kg i.p., applied daily for 5 days) appreciably decreased the number of injured pyramidal cells from 1850+/-100/mm(2) to 1000+/-300/mm(2) (p<0.00...

journal_title：Neurobiology of disease

authors： Sas K,Robotka H,Rózsa E,Agoston M,Szénási G,Gigler G,Marosi M,Kis Z,Farkas T,Vécsei L,Toldi J

更新日期：2008-11-01 00:00:00

abstract：:Huntington's disease (HD) is one of the few neurodegenerative diseases with a known genetic cause, knowledge that has enabled the creation of animal models using genetic manipulations that aim to recapitulate HD pathology. The study of behavioral and neuropathological phenotypes of these HD models, however, has been p...

journal_title：Neurobiology of disease

authors： Menalled L,El-Khodor BF,Patry M,Suárez-Fariñas M,Orenstein SJ,Zahasky B,Leahy C,Wheeler V,Yang XW,MacDonald M,Morton AJ,Bates G,Leeds J,Park L,Howland D,Signer E,Tobin A,Brunner D

更新日期：2009-09-01 00:00:00

abstract：:Fetal alcohol exposure causes severe neuropsychiatric problems, but mechanisms of the ethanol-associated changes in central nervous system development are unclear. In vivo, ethanol's interaction with N-methyl-D-aspartate (NMDA) and gamma-aminobutyric acid type A (GABA(A)) receptors may cause increased apoptosis in the...

journal_title：Neurobiology of disease

authors： Moulder KL,Fu T,Melbostad H,Cormier RJ,Isenberg KE,Zorumski CF,Mennerick S

更新日期：2002-08-01 00:00:00

abstract：:Uridine, like adenosine, is released under sustained depolarization and it can inhibit hippocampal neuronal activity, suggesting that uridine may be released during seizures and can be involved in epileptic mechanisms. In an in vivo microdialysis study, we measured the extracellular changes of nucleoside and amino aci...

journal_title：Neurobiology of disease

authors： Slézia A,Kékesi AK,Szikra T,Papp AM,Nagy K,Szente M,Maglóczky Z,Freund TF,Juhász G

更新日期：2004-08-01 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：:Excitotoxicity plays a major role in the pathogenesis of Huntington disease (HD), a fatal neurodegenerative disorder. Adenosine A(2A) receptors (A(2A)Rs) modulate excitotoxicity and have been suggested to play a pathogenetic role in HD. The main aim of this study was to evaluate the effect of A(2A)R blockade on the ex...

journal_title：Neurobiology of disease

authors： Martire A,Ferrante A,Potenza RL,Armida M,Ferretti R,Pézzola A,Domenici MR,Popoli P

更新日期：2010-01-01 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：: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