Pharmacogenetic modulation of STEP improves motor and cognitive function in a mouse model of Huntington's disease.

abstract：:Huntingtin, the protein product of the Huntington's disease (HD) gene, is known to interact with the tumor suppressor p53. It has recently been shown that activation of p53 upregulates the level of huntingtin, both in vitro and in vivo, whereas p53 deficiency in HD-transgenic flies and mice has been found to be benefi...

journal_title：Neurobiology of disease

authors： Ryan AB,Zeitlin SO,Scrable H

更新日期：2006-11-01 00:00:00

abstract：:β-Amyloid (Aβ) plaques in Alzheimer (AD) brains are surrounded by severe dendritic and axonal changes, including local spine loss, axonal swellings and distorted neurite trajectories. Whether and how plaques induce these neuropil abnormalities remains unknown. We tested the hypothesis that oligomeric assemblies of Aβ,...

journal_title：Neurobiology of disease

authors： DaRocha-Souto B,Coma M,Pérez-Nievas BG,Scotton TC,Siao M,Sánchez-Ferrer P,Hashimoto T,Fan Z,Hudry E,Barroeta I,Serenó L,Rodríguez M,Sánchez MB,Hyman BT,Gómez-Isla T

更新日期：2012-01-01 00:00:00

abstract：:Dystonias are movement disorders whose pathomechanism is largely unknown. Dystonic dt(sz) hamsters represent a model of primary dystonias, where alterations of striatal interneuron density and sodium channel function in projection neurones were described. Here, using cortico-striatal slices, we explore whether also th...

journal_title：Neurobiology of disease

authors： Köhling R,Koch UR,Hamann M,Richter A

更新日期：2004-06-01 00:00:00

abstract：:Frontotemporal dementia (FTD) is typified by behavioral and cognitive changes manifested as altered social comportment and impaired memory performance. To investigate the neurodegenerative consequences of progranulin gene (GRN) mutations, which cause an inherited form of FTD, we used previously generated progranulin k...

journal_title：Neurobiology of disease

authors： Ghoshal N,Dearborn JT,Wozniak DF,Cairns NJ

更新日期：2012-01-01 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：:TAR-DNA binding protein 43 (TDP-43) is a multifunctional RNA binding protein directly implicated in the etiology of amyotrophic lateral sclerosis (ALS). Previous studies have demonstrated that loss of TDP-43 function leads to intracellular accumulation of non-coding repetitive element transcripts and double-stranded R...

journal_title：Neurobiology of disease

authors： LaRocca TJ,Mariani A,Watkins LR,Link CD

更新日期：2019-12-01 00:00:00

abstract：:Insulin deficiency may contribute toward the neurological deficits of diabetic polyneuropathy (DPN). In particular, the unique trophic properties of insulin, acting on sensory neuron and axon receptors offer an approach toward reversing loss of skin axons that develops during diabetes. Here we examined how local cutan...

journal_title：Neurobiology of disease

authors： Guo G,Kan M,Martinez JA,Zochodne DW

更新日期：2011-08-01 00:00:00

abstract：:The effects of an adenovirus-mediated Bcl-X(L) expression, driven by a neuron-specific human synapsin-1 promoter, on the degree of injury, were examined after transient focal ischemia in mice. Therefore, injections of vehicle, of an adenoviral E1-deleted control vector (Ad-dE1), or a Bcl-X(L) vector (Ad-Syn-Bcl-X(L)) ...

journal_title：Neurobiology of disease

authors： Kilic E,Hermann DM,Kügler S,Kilic U,Holzmüller H,Schmeer C,Bähr M

更新日期：2002-11-01 00:00:00

abstract：:The impact of Abeta deposition upon cholinergic intrinsic cortical and striatal, as well as basal forebrain long projection neuronal systems was qualitatively and quantitatively evaluated in young (2-6 months) and middle-aged (10-16 months) APPswe/PS1DeltaE9 transgenic (tg) mice. Cholinergic neuritic swellings occurre...

journal_title：Neurobiology of disease

authors： Perez SE,Dar S,Ikonomovic MD,DeKosky ST,Mufson EJ

更新日期：2007-10-01 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：:The molecular mechanisms of major mental illnesses, such as schizophrenia and bipolar disorder, are unclear. To address this fundamental question, many groups have studied molecular expression profiles in postmortem brains and other tissues from patients compared with those from normal controls. Development of unbiase...

journal_title：Neurobiology of disease

authors： Lin CY,Sawa A,Jaaro-Peled H

更新日期：2012-01-01 00:00:00

abstract：:The Alzheimer disease-associated beta-amyloid peptide has been shown to induce apoptotic neuronal death. In the present study, we test the hypothesis that the apoptotic pathway activated by beta-amyloid is similar to the pathway activated by the Fas/TNFR family of death receptors, which requires caspase-8 activity and...

journal_title：Neurobiology of disease

authors： Ivins KJ,Thornton PL,Rohn TT,Cotman CW

更新日期：1999-10-01 00:00:00

abstract：:Prion strains yield specific neuropathological features including spongiform degeneration and deposition patterns of pathological prion protein. Their invariant regional distribution, following variations in the infection route, has led to the proposal that prions replicate preferentially in defined neuro-anatomical a...

journal_title：Neurobiology of disease

authors： Langevin C,Andréoletti O,Le Dur A,Laude H,Béringue V

更新日期：2011-01-01 00:00:00

abstract：:Apoptosis is triggered in the developing mammalian brain by sedative, anesthetic or antiepileptic drugs during late gestation and early life. Whether human children are vulnerable to this toxicity mechanism remains unknown, as there are no imaging techniques to capture it. Apoptosis is characterized by distinct struct...

journal_title：Neurobiology of disease

authors： Rosado-Mendez IM,Noguchi KK,Castañeda-Martinez L,Kirvassilis G,Wang SH,Manzella F,Swiney BS,Masuoka K,Capuano S III2,Brunner KG,Crosno K,Guerrero QW,Whitson H,Brambrink A,Simmons HS,Mejia AF,Zagzebski JA,Hall TJ,Ikono

更新日期：2019-07-01 00:00:00

abstract：:Attention deficit hyperactivity disorder (ADHD) is characterized by hyperactivity, inattention, and impulsivity. The coloboma mouse model of ADHD exhibits profound hyperactivity. To determine whether coloboma mice exhibit other signs of ADHD, we assessed latent inhibition as a test of attention, and impulsivity in a d...

journal_title：Neurobiology of disease

authors： Bruno KJ,Freet CS,Twining RC,Egami K,Grigson PS,Hess EJ

更新日期：2007-01-01 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：:Several studies indicate the involvement of advanced glycation end-products (AGEs) in neurodegenerative diseases. Moreover, the rising consumption of fructose in industrialized countries has been related to cognitive impairment, but the impact of fructose-derived AGEs on hippocampus has never been investigated. The pr...

journal_title：Neurobiology of disease

authors： Mastrocola R,Nigro D,Cento AS,Chiazza F,Collino M,Aragno M

更新日期：2016-05-01 00:00:00

abstract：:Frontotemporal dementia (FTD) is one of the most prevalent forms of early-onset dementia. It represents part of the FTD-Amyotrophic Lateral Sclerosis (ALS) spectrum, a continuum of genetically and pathologically overlapping disorders. FTD-causing mutations in CHMP2B, a gene encoding a core component of the heteromeric...

journal_title：Neurobiology of disease

authors： West RJH,Ugbode C,Fort-Aznar L,Sweeney ST

更新日期：2020-10-01 00:00:00

abstract：:In the present study, we prepared a SCA3 animal model by generating transgenic mice expressing polyglutamine-expanded ataxin-3-Q79. Ataxin-3-Q79 was expressed in brain areas implicated in SCA3 neurodegeneration, including cerebellum, pontine nucleus and substantia nigra. Ataxin-3-Q79 transgenic mice displayed motor dy...

journal_title：Neurobiology of disease

authors： Chou AH,Yeh TH,Ouyang P,Chen YL,Chen SY,Wang HL

更新日期：2008-07-01 00:00:00

abstract：:Mesenchymal stem cells (MSCs) are reported to possess immunomodulatory properties. Previous reports have demonstrated the beneficial effects of MSC-transplantation in focal cerebral ischemia animal models. In this study, we have investigated the neuroimmunomodulatory functions of human MSCs, transplanted in a rat foca...

journal_title：Neurobiology of disease

authors： Sheikh AM,Nagai A,Wakabayashi K,Narantuya D,Kobayashi S,Yamaguchi S,Kim SU

更新日期：2011-03-01 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：: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：:Huntington disease (HD) is an adult onset, neurodegenerative disorder that results from CAG expansion in the HD gene. Recent work has demonstrated testicular degeneration in mouse models of HD and alterations in the hypothalamic-pituitary-gonadal (HPG) axis in HD patients. Here, we show that HD patients have specific ...

journal_title：Neurobiology of disease

authors： Van Raamsdonk JM,Murphy Z,Selva DM,Hamidizadeh R,Pearson J,Petersén A,Björkqvist M,Muir C,Mackenzie IR,Hammond GL,Vogl AW,Hayden MR,Leavitt BR

更新日期：2007-06-01 00:00:00

abstract：:Neurofibrillary degeneration in transgenic models of tauopathies has been observed to be enhanced when these models are crossed with transgenic models developing an Aβ pathology. The mechanisms leading to this enhanced tau pathology are not well understood. We have performed a detailed analysis of tau misprocessing in...

journal_title：Neurobiology of disease

authors： Héraud C,Goufak D,Ando K,Leroy K,Suain V,Yilmaz Z,De Decker R,Authelet M,Laporte V,Octave JN,Brion JP

更新日期：2014-02-01 00:00:00

abstract：:Experiments reported here were motivated by studies in both human epilepsy and animal models in which stunted dendritic arbors are observed. Our goal was to determine if chronic network hyperexcitability alters dendritic growth. Experiments were conducted in hippocampal slice cultures obtained from infant mice that ex...

journal_title：Neurobiology of disease

authors： Nishimura M,Owens J,Swann JW

更新日期：2008-02-01 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：: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：: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：:Spinocerebellar ataxia type 3 (SCA3) or Machado-Joseph disease (MJD) belongs to a group of autosomal dominant neurodegenerative diseases, which are caused by the expansion of a polyglutamine repeat in the affected protein, in this case ataxin-3. Ataxin-3 is mainly localized in the cytoplasm; however, one hallmark of S...

journal_title：Neurobiology of disease

authors： Antony PM,Mäntele S,Mollenkopf P,Boy J,Kehlenbach RH,Riess O,Schmidt T

更新日期：2009-11-01 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