Reduced post-stroke glial scarring in the infant primate brain reflects age-related differences in the regulation of astrogliosis.

abstract：:Fragile X Syndrome (FXS) is the most common inherited cause of intellectual disability, and is the leading known single-gene cause of autism spectrum disorder. FXS patients display varied behavioural deficits that include mild to severe cognitive impairments in addition to mood disorders. Currently there is no cure fo...

journal_title：Neurobiology of disease

authors： Yau SY,Bettio L,Vetrici M,Truesdell A,Chiu C,Chiu J,Truesdell E,Christie BR

更新日期：2018-05-01 00:00:00

abstract：:The mutilated-foot rat (mf rat) is an autosomal recessive mutant with characteristic digit deformities in adult animals, and this phenotype mimics many aspects of human sensory neuropathy. The genetics of mf rats was recently elucidated. To understand whether the genotype is responsible for cutaneous denervation befor...

journal_title：Neurobiology of disease

authors： Hsu SH,Lee MJ,Hsieh SC,Scaravilli F,Hsieh ST

更新日期：2004-07-01 00:00:00

abstract：:Tissue transglutaminase (tTG) is an indicator of acute cell death in vitro. An increase in tTG protein level is found in postmortem Alzheimer's disease (AD) brains as well as in Huntington's disease. No study revealed tTG in vivo so far. We investigated the concentrations of tTG in the cerebrospinal fluid (CSF) obtain...

journal_title：Neurobiology of disease

authors： Bonelli RM,Aschoff A,Niederwieser G,Heuberger C,Jirikowski G

更新日期：2002-10-01 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

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：: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：:Sex differences are a well-known phenomenon in Alzheimer's disease (AD), with women having a higher risk for AD than men. Many AD mouse models display a similar sex-dependent pattern, with females showing earlier cognitive deficits and more severe neuropathology than males. However, whether those differences are relev...

journal_title：Neurobiology of disease

authors： Melnikova T,Park D,Becker L,Lee D,Cho E,Sayyida N,Tian J,Bandeen-Roche K,Borchelt DR,Savonenko AV

更新日期：2016-12-01 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：:To explore mechanisms of epileptogenesis in audiogenic seizures (AGS), we examined the expression of alpha-amino-3-hydroxy-5-methyl-4-isoxazoleopropionic acid (AMPA) receptor subunits GluR1 and GluR2 and of the GluR-associated protein Narp in the hippocampus and the inferior colliculus (IC) from AGS-susceptible P77PMC...

journal_title：Neurobiology of disease

authors： Li SY,Xu DS,Jia HT

更新日期：2003-12-01 00:00:00

abstract：:The choroid plexus contains a major reservoir of macrophages poised for efficient delivery of virus and neurotoxins to the brain after infection by lentiviruses such as human or feline immunodeficiency virus (FIV). However, their contribution to neurotoxicity is poorly understood. Medium from FIV-infected, choroid ple...

journal_title：Neurobiology of disease

authors： Bragg DC,Boles JC,Meeker RB

更新日期：2002-03-01 00:00:00

abstract：:Multiple lines of evidence implicate striatal dysfunction in the pathogenesis of dystonia, including in DYT1, a common inherited form of the disease. The impact of striatal dysfunction on connected motor circuits and their interaction with other brain regions is poorly understood. Conditional knock-out (cKO) of the DY...

journal_title：Neurobiology of disease

authors： DeSimone JC,Pappas SS,Febo M,Burciu RG,Shukla P,Colon-Perez LM,Dauer WT,Vaillancourt DE

更新日期：2017-10-01 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：: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：:The pathophysiology of Parkinson's disease (PD) and of L-DOPA-induced dyskinesia (LID) is associated with dysfunctional neuronal activity in several nuclei of the basal ganglia. Moreover, high levels of oscillatory activity and synchronization have also been described in both intra- and inter-basal ganglia nuclei and ...

journal_title：Neurobiology of disease

authors： Aristieta A,Ruiz-Ortega JA,Miguelez C,Morera-Herreras T,Ugedo L

更新日期：2016-05-01 00:00:00

abstract：:The epsilon4 allele of the apolipoprotein E gene (APOE) is associated with sporadic and familial late-onset Alzheimer's disease (AD). Oxidative stress is believed to play an important role in neuronal dysfunction and cell death in AD. We now provide evidence that in the hippocampus of AD, the level of thiobarbituric a...

journal_title：Neurobiology of disease

authors： Ramassamy C,Averill D,Beffert U,Theroux L,Lussier-Cacan S,Cohn JS,Christen Y,Schoofs A,Davignon J,Poirier J

更新日期：2000-02-01 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：:Butyrylcholinesterase (BChE) genotypes and protein (BuChE) activity, especially in combination with Apolipoprotein E4 (ApoE4), have been investigated as risk factors for developing Alzheimer disease (AD) and may be associated with the rate of progression of cognitive decline. Despite similar pathologic (e.g. amyloid d...

journal_title：Neurobiology of disease

authors： Maetzler W,Keller S,Michelis J,Koehler N,Stransky E,Becker C,Schulte C,Melms A,Gasser T,Berg D

更新日期：2009-08-01 00:00:00

abstract：:We identify an essential role for the RGD (Arg-Gly-Asp tripeptide) moiety in vivo during adult peripheral neuron regenerative growth. Beyond a peripheral nerve transection there were rises in the fibronectin extracellular matrix, and striking rises in the mRNA and protein expression of integrin subunits sensitive to R...

journal_title：Neurobiology of disease

authors： Liu WQ,Martinez JA,Durand J,Wildering W,Zochodne DW

更新日期：2009-04-01 00:00:00

abstract：BACKGROUND:Alzheimer's disease (AD) is the most common neurodegenerative disorder. Depositions of amyloid β peptide (Aβ) and tau protein are among the major pathological hallmarks of AD. Aβ and tau burden follows predictable spatial patterns during the progression of AD. Nevertheless, it remains obscure why certain bra...

journal_title：Neurobiology of disease

authors： Mendonça CF,Kuras M,Nogueira FCS,Plá I,Hortobágyi T,Csiba L,Palkovits M,Renner É,Döme P,Marko-Varga G,Domont GB,Rezeli M

更新日期：2019-10-01 00:00:00

abstract：:The use of energy substrates, such as lactate and pyruvate, has been shown to improve synaptic function when administered during glucose deprivation. In the present study, we investigated whether prolonged incubation with monocarboxylate (pyruvate or lactate) prior rather than during glucose deprivation can also susta...

journal_title：Neurobiology of disease

authors： Shetty PK,Sadgrove MP,Galeffi F,Turner DA

更新日期：2012-01-01 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：:Amino-terminal fragments of huntingtin (htt) appear to result from proteolytic processing of the full-length protein in Huntington's disease (HD), and fragments containing pathological expansions of polyglutamine elicit toxicity in model systems. Such fragments are sequestered into insoluble aggregates, which may init...

journal_title：Neurobiology of disease

authors： Miller TW,Zhou C,Gines S,MacDonald ME,Mazarakis ND,Bates GP,Huston JS,Messer A

更新日期：2005-06-01 00:00:00

abstract：BACKGROUND/INTRODUCTION:The neuroinflammatory response plays a major role in the secondary injury cascade after traumatic spinal cord injury (SCI). To date, systemic anti-inflammatory medications such as methylprednisolone sodium succinate (MPSS) have shown promise in SCI. However, systemic immunosuppression can have d...

journal_title：Neurobiology of disease

authors： Chio JCT,Wang J,Surendran V,Li L,Zavvarian MM,Pieczonka K,Fehlings MG

更新日期：2021-01-01 00:00:00

abstract：:Presenilin 1 (PSEN1) mutations are the main cause of autosomal dominant Early-onset Alzheimer Disease (EOAD). Among them, deletions of exon 9 have been reported to be associated with a phenotype of spastic paraparesis. Using exome data from a large sample of 522 EOAD cases and 584 controls to search for genomic copy-n...

journal_title：Neurobiology of disease

authors： Le Guennec K,Veugelen S,Quenez O,Szaruga M,Rousseau S,Nicolas G,Wallon D,Fluchere F,Frébourg T,De Strooper B,Campion D,Chávez-Gutiérrez L,Rovelet-Lecrux A

更新日期：2017-08-01 00:00:00

abstract：:The interest in the pedunculopontine tegmental nucleus (PPTg), a structure located in the brainstem at the level of the pontomesencephalic junction, has greatly increased in recent years because it is involved in the regulation of physiological functions that fail in Parkinson's disease and because it is a promising t...

journal_title：Neurobiology of disease

authors： Vitale F,Capozzo A,Mazzone P,Scarnati E

更新日期：2019-08-01 00:00:00

abstract：:Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder characterized behaviorally by chorea, incoordination, and shortened lifespan and neuropathologically by huntingtin inclusions and neuronal degeneration. In order to facilitate studies of pathogenesis and therapeutics, we have generated a new...

journal_title：Neurobiology of disease

authors： Tanaka Y,Igarashi S,Nakamura M,Gafni J,Torcassi C,Schilling G,Crippen D,Wood JD,Sawa A,Jenkins NA,Copeland NG,Borchelt DR,Ross CA,Ellerby LM

更新日期：2006-02-01 00:00:00

abstract：:Dystonia and levodopa-induced dyskinesia (LID) are both hyperkinetic movement disorders. Dystonia arises most often spontaneously, although it may be seen after stroke, injury, or as a result of genetic causes. LID is associated with Parkinson's disease (PD), emerging as a consequence of chronic therapy with levodopa,...

journal_title：Neurobiology of disease

authors： Calabresi P,Standaert DG

更新日期：2019-12-01 00:00:00

abstract：:Friedreich ataxia (FRDA) is an autosomal recessive degenerative disease caused either by an intronic GAA triplet repeat expansion that suppresses the expression of the frataxin gene on chromosome 9q13, or, rarely, by point mutations in the frataxin gene. We investigated the expression of the mouse frataxin homologue d...

journal_title：Neurobiology of disease

authors： Jiralerspong S,Liu Y,Montermini L,Stifani S,Pandolfo M

更新日期：1997-01-01 00:00:00

abstract：:During the last few years, adenoviral gene transfer techniques have achieved increasing interest in the treatment of neurodegenerative diseases. However, gene therapy requires that delivered genes are translated into proteins. This may pose a problem in focal ischemia where protein synthesis is compromized. The presen...

journal_title：Neurobiology of disease

authors： Hermann DM,Kilic E,Kügler S,Isenmann S,Bähr M

更新日期：2001-08-01 00:00:00

abstract：:Exposure to stress during pregnancy influences the trajectory of brain development resulting in permanent alterations that may contribute to increased susceptibility to subsequent cognitive or neuropsychiatric disorders. In this manuscript, we examined the effects of prenatal stress on the expression of basic fibrobla...

journal_title：Neurobiology of disease

authors： Fumagalli F,Bedogni F,Slotkin TA,Racagni G,Riva MA

更新日期：2005-12-01 00:00:00