Role of CSPG receptor LAR phosphatase in restricting axon regeneration after CNS injury.

abstract：:TRIM family proteins are involved in a broad range of biological processes, and their alteration results in many diverse pathological conditions found in genetic diseases, viral infections, and cancers. However, the spatial and temporal expression and function of TRIM9, one of TRIM family proteins, remain obscure. Our...

journal_title：Neurobiology of disease

authors： Tanji K,Kamitani T,Mori F,Kakita A,Takahashi H,Wakabayashi K

更新日期：2010-05-01 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：BACKGROUND:Epidemiological studies suggest that the risk of neurodevelopmental disorders such as autism spectrum disorder (ASD) and schizophrenia is increased by prenatal exposure to viral or bacterial infection during pregnancy. It is still unclear how activation of the maternal immune response interacts with underlyi...

journal_title：Neurobiology of disease

authors： Amodeo DA,Lai CY,Hassan O,Mukamel EA,Behrens MM,Powell SB

更新日期：2019-05-01 00:00:00

abstract：:Mutations in the DNA-binding domain of EGR2 are associated with severe autosomal dominant forms of peripheral neuropathy. In this study, we show that one such Egr2 mutant (S382R, D383Y), when expressed in Schwann cells in vitro, is not transcriptionally inactive but retains residual wild-type Egr2 functions, including...

journal_title：Neurobiology of disease

authors： Arthur-Farraj P,Mirsky R,Parkinson DB,Jessen KR

更新日期：2006-10-01 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：:The rapidly emerging science of epigenetics and epigenomic medicine promises to reveal novel insights into the susceptibility to and the onset and progression of epileptic disorders. Epigenetic regulatory mechanisms are now implicated in orchestrating aspects of neural development (e.g., cell fate specification and ma...

journal_title：Neurobiology of disease

authors： Qureshi IA,Mehler MF

更新日期：2010-07-01 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：: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：: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：: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：:Huntington's disease (HD) is a late-onset neurodegenerative disease for which the mutation is CAG/polyglutamine repeat expansion. The R6 mouse lines expressing the HD mutation develop a movement disorder that is preceded by the formation of neuronal polyglutamine aggregates. The phenotype is likely caused by a widespr...

journal_title：Neurobiology of disease

authors： Liévens JC,Woodman B,Mahal A,Spasic-Boscovic O,Samuel D,Kerkerian-Le Goff L,Bates GP

更新日期：2001-10-01 00:00:00

abstract：:Alzheimer's disease (AD) is characterized by overproduction of A beta derived from APP cleavage via beta- and gamma-secretase pathway. Recent evidence has linked altered cholesterol metabolism to AD pathogenesis. In this study, we show that AD brain had significant cholesterol retention and high beta- and gamma-secret...

journal_title：Neurobiology of disease

authors： Xiong H,Callaghan D,Jones A,Walker DG,Lue LF,Beach TG,Sue LI,Woulfe J,Xu H,Stanimirovic DB,Zhang W

更新日期：2008-03-01 00:00:00

abstract：:There is clear evidence that an inflammatory reaction is mounted within the CNS following trauma, stroke, infection and seizures, thus augmenting brain damage. Furthermore, chronic inflammation of the CNS is implicated in many neurodegenerative disorders. However, the effects of products of inflammation on neuronal ce...

journal_title：Neurobiology of disease

authors： Ogburn KD,Bottiglieri T,Wang Z,Figueiredo-Pereira ME

更新日期：2006-05-01 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：:Altered expression and distribution of neurotransmitter receptors, including metabotropic glutamate receptors (mGluRs), constitute key aspects in epileptogenesis, impaired hippocampal excitability and neuronal degeneration. mGluR1 mediates predominantly excitatory effects, whereas mGluR4 acts as inhibitory presynaptic...

journal_title：Neurobiology of disease

authors： Pitsch J,Schoch S,Gueler N,Flor PJ,van der Putten H,Becker AJ

更新日期：2007-06-01 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：: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

abstract：:Drugs that modify noradrenergic transmission such as atomoxetine and clonidine are increasingly prescribed for the treatment of attention deficit hyperactivity disorder (ADHD). However, the therapeutic targets of these compounds are unknown. Norepinephrine is also implicated in the hyperactivity exhibited by coloboma ...

journal_title：Neurobiology of disease

authors： Bruno KJ,Hess EJ

更新日期：2006-09-01 00:00:00

abstract：:Infection of immunocompetent adult rats with Borna disease virus (BDV) causes severe encephalitis and neural dysfunction. The expression of COX-2 and CGRP, genes previously shown to be implicated in CNS disease and peripheral inflammation, was dramatically upregulated in the cortical neurons of acutely BDV-infected ra...

journal_title：Neurobiology of disease

authors： Röhrenbeck AM,Bette M,Hooper DC,Nyberg F,Eiden LE,Dietzschold B,Weihe E

更新日期：1999-02-01 00:00:00

abstract：:Extracellular nucleotides exert a variety of biological actions through different subtypes of P2 receptors. Here we characterized in the human neuroblastoma SH-SY5Y cells the simultaneous presence of various P2 receptors, belonging to the P2X ionotropic and P2Y metabotropic families. Western blot analysis detected the...

journal_title：Neurobiology of disease

authors： Cavaliere F,Nestola V,Amadio S,D'Ambrosi N,Angelini DF,Sancesario G,Bernardi G,Volonté C

更新日期：2005-02-01 00:00:00

abstract：:Motor neuron degeneration in amyotrophic lateral sclerosis (ALS) is a form of apoptosis, but the mechanisms for this neuronal cell death are not known. We evaluated whether motor neuron degeneration in ALS is associated with changes in the levels and function of the apoptosis regulating protein p53. The protein levels...

journal_title：Neurobiology of disease

authors： Martin LJ

更新日期：2000-12-01 00:00:00

abstract：:We identified a novel spontaneous mutant mouse showing motor symptoms that are similar to those of the dystonia musculorum (dt) mouse. The observations suggested that the mutant mice inherited the mild dt phenotype as an autosomal recessive trait. Linkage analysis showed that the causative gene was located near D1Mit3...

journal_title：Neurobiology of disease

authors： Horie M,Mekada K,Sano H,Kikkawa Y,Chiken S,Someya T,Saito K,Hossain MI,Nameta M,Abe K,Sakimura K,Ono K,Nambu A,Yoshiki A,Takebayashi H

更新日期：2016-12-01 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：:Intermittent hypoxia (IH) during sleep induces temporally defined increases in apoptosis within vulnerable brain regions such as the hippocampal CA1 region in rats. Protein kinase B (AKT) has emerged as major signal transduction protein underlying inhibition of apoptosis and consequent increases in cell survival. Spra...

journal_title：Neurobiology of disease

authors： Goldbart A,Cheng ZJ,Brittian KR,Gozal D

更新日期：2003-12-01 00:00:00

abstract：:The death of mature oligodendrocytes (OLs) leads to demyelination in the central nervous system (CNS) and subsequently to functional deficits. Remyelination requires the differentiation of oligodendrocyte progenitor cells (OPCs) into myelinating OLs, which in the CNS with neurodegenerative diseases such as multiple sc...

journal_title：Neurobiology of disease

authors： Zhang J,Zhang ZG,Lu M,Zhang Y,Shang X,Chopp M

更新日期：2019-05-01 00:00:00

abstract：:The blossoming field of epitranscriptomics has recently garnered attention across many fields by findings that chemical modifications on RNA have immense biological consequences. Methylation of nucleotides in RNA, including N6-methyladenosine (m6A), 2-O-dimethyladenosine (m6Am), N1-methyladenosine (m1A), 5-methylcytos...

journal_title：Neurobiology of disease

authors： Vissers C,Sinha A,Ming GL,Song H

更新日期：2020-12-01 00:00:00

abstract：:Dietary or pharmacological manipulation of plasma lipids markedly influences amyloid deposition in animal models of Alzheimer's Disease (AD). However, it is not known whether baseline plasma lipids in AD models differ from wild-type littermates throughout the natural history of disease. To address this question, we me...

journal_title：Neurobiology of disease

authors： Burgess BL,McIsaac SA,Naus KE,Chan JY,Tansley GH,Yang J,Miao F,Ross CJ,van Eck M,Hayden MR,van Nostrand W,St George-Hyslop P,Westaway D,Wellington CL

更新日期：2006-10-01 00:00:00

abstract：:DYT1 dystonia is an inherited disease linked to mutation in the TOR1A gene encoding for the protein torsinA. Although the mechanism by which this genetic alteration leads to dystonia is unclear, multiple lines of clinical evidence suggest a link between dystonia and a reduced dopamine D2 receptor (D2R) availability. B...

journal_title：Neurobiology of disease

authors： Napolitano F,Pasqualetti M,Usiello A,Santini E,Pacini G,Sciamanna G,Errico F,Tassone A,Di Dato V,Martella G,Cuomo D,Fisone G,Bernardi G,Mandolesi G,Mercuri NB,Standaert DG,Pisani A

更新日期：2010-06-01 00:00:00

abstract：:In multiple sclerosis (MS), post-mortem studies of human brain tissue as well as data from animal models have shown that apoptosis of neurons occurs to a significant extent during this disease. As neurodegeneration in MS correlates with permanent neurological deficits in patients, understanding the mechanisms would be...

journal_title：Neurobiology of disease

authors： Diem R,Taheri N,Dietz GP,Kuhnert A,Maier K,Sättler MB,Gadjanski I,Merkler D,Bähr M

更新日期：2005-11-01 00:00:00

abstract：:Neurodegenerative diseases impose a burden on society, yet for the most part, the mechanisms underlying neuronal dysfunction and death in these disorders remain unclear despite the identification of relevant disease genes. Given the molecular conservation in neuronal signaling pathways across vertebrate and invertebra...

journal_title：Neurobiology of disease

authors： Dimitriadi M,Hart AC

更新日期：2010-10-01 00:00:00