Development of an aggregate-selective, human-derived α-synuclein antibody BIIB054 that ameliorates disease phenotypes in Parkinson's disease models.

abstract：:Photoreceptor cell death is an irreversible, pathologic event in many blinding retinal diseases including retinitis pigmentosa, age-related macular disease, and retinal detachment. Light exposure can exacerbate a variety of human retinal diseases by increasing the rate of photoreceptor cell death. In the present study...

journal_title：Neurobiology of disease

authors： Kong L,Li F,Soleman CE,Li S,Elias RV,Zhou X,Lewis DA,McGinnis JF,Cao W

更新日期：2006-03-01 00:00:00

abstract：:Although peroxisome biogenesis and β-oxidation disorders are well known for their neurodevelopmental defects, patients with these disorders are increasingly diagnosed with neurodegenerative pathologies. In order to investigate the cellular mechanisms of neurodegeneration in these patients, we developed a mouse model l...

journal_title：Neurobiology of disease

authors： Verheijden S,Bottelbergs A,Krysko O,Krysko DV,Beckers L,De Munter S,Van Veldhoven PP,Wyns S,Kulik W,Nave KA,Ramer MS,Carmeliet P,Kassmann CM,Baes M

更新日期：2013-10-01 00:00:00

abstract：:An important aspect of Huntington's disease (HD) pathogenesis which may have important therapeutic implications is that the cellular events leading to cell death may be different in cortical and striatal neurons. In the present study, we characterized cellular changes in cortical and striatal neurons treated with the ...

journal_title：Neurobiology of disease

authors： Galas MC,Bizat N,Cuvelier L,Bantubungi K,Brouillet E,Schiffmann SN,Blum D

更新日期：2004-02-01 00:00:00

abstract：:Mental illness is the leading cause of disability worldwide. We are only just beginning to reveal and comprehend the complex interaction that exists between the genetic makeup of an organism and the potential modifying effect of the environment in which it lives, and how this translates into mediating susceptibility t...

journal_title：Neurobiology of disease

authors： Renoir T,Pang TY,Hannan AJ

更新日期：2013-09-01 00:00:00

abstract：:No single-omic approach completely elucidates the multitude of alterations taking place in Alzheimer's disease (AD). Here, we coupled transcriptomic and phosphoproteomic approaches to determine the temporal sequence of changes in mRNA, protein, and phosphopeptide expression levels from human temporal cortical samples,...

journal_title：Neurobiology of disease

authors： Marttinen M,Paananen J,Neme A,Mitra V,Takalo M,Natunen T,Paldanius KMA,Mäkinen P,Bremang M,Kurki MI,Rauramaa T,Leinonen V,Soininen H,Haapasalo A,Pike I,Hiltunen M

更新日期：2019-04-01 00:00:00

abstract：:Senile plaques composed mainly of beta-amyloid (Abeta) and neurofibrillary tangles principally composed of hyperphosphorylated tau are the major pathological features of Alzheimer's disease (AD). Despite the fact that increased expression of amyloid precursor protein (APP) and presenilin-1 (PS1) transgenes in mice lea...

journal_title：Neurobiology of disease

authors： Kurt MA,Davies DC,Kidd M,Duff K,Howlett DR

更新日期：2003-10-01 00:00:00

abstract：:Recent studies indicate that the Parkinson's disease-linked leucine-rich repeat kinase 2 (LRRK2) modulates cytoskeletal functions by regulating actin and tubulin dynamics, thereby affecting neurite outgrowth. By interactome analysis we demonstrate that the binding of LRRK2 to tubulins is significantly enhanced by phar...

journal_title：Neurobiology of disease

authors： Caesar M,Zach S,Carlson CB,Brockmann K,Gasser T,Gillardon F

更新日期：2013-06-01 00:00:00

abstract：:Copper is an essential trace metal which plays a fundamental role in the biochemistry of the human nervous system. Menkes disease and Wilson disease are inherited disorders of copper metabolism and the dramatic neurodegenerative phenotypes of these two diseases underscore the essential nature of copper in nervous syst...

journal_title：Neurobiology of disease

authors： Waggoner DJ,Bartnikas TB,Gitlin JD

更新日期：1999-08-01 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 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：:Rubrospinal neurons (RSNs) undergo marked atrophy after cervical axotomy. This progressive atrophy may impair the regenerative capacity of RSNs in response to repair strategies that are targeted to promote rubrospinal tract regeneration. Here, we investigated whether we could achieve long-term rescue of RSNs from lesi...

journal_title：Neurobiology of disease

authors： Ruitenberg MJ,Blits B,Dijkhuizen PA,te Beek ET,Bakker A,van Heerikhuize JJ,Pool CW,Hermens WT,Boer GJ,Verhaagen J

更新日期：2004-03-01 00:00:00

abstract：:Based on in vitro studies, it is hypothesized that neurotrophic factor deprivation following deafferentation elicits an oxidative state change in the deafferented neuron and the formation of free radicals that then signal cell death pathways. This pathway to cell death was tested in vivo by assessing the efficacy of a...

journal_title：Neurobiology of disease

authors： Maruyama J,Yamagata T,Ulfendahl M,Bredberg G,Altschuler RA,Miller JM

更新日期：2007-02-01 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：:Malfunction of the ubiquitin-proteasome system has been implicated as a causal factor in the pathogenesis of aggregation-related disorders, e.g. Parkinson's disease. We show here that Transforming growth factor-beta 1 (TGF-beta), a multifunctional cytokine and trophic factor for dopaminergic (DAergic) neurons modulate...

journal_title：Neurobiology of disease

authors： Knöferle J,Ramljak S,Koch JC,Tönges L,Asif AR,Michel U,Wouters FS,Heermann S,Krieglstein K,Zerr I,Bähr M,Lingor P

更新日期：2010-06-01 00:00:00

abstract：:Huntington's disease (HD) is a neurodegenerative disorder involving progressive motor disturbances, cognitive decline, and desynchronized sleep-wake rhythms. Recent studies revealed that restoring normal sleep-wake cycles can improve cognitive function in HD mice, suggesting that some sleep/wake systems remain operati...

journal_title：Neurobiology of disease

authors： Williams RH,Morton AJ,Burdakov D

更新日期：2011-06-01 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：: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：:In Alzheimer's disease (AD), the cognitive reserve theory predicts that at any level of assessed clinical severity, the underlying brain pathology is more advanced in patients with more cognitive reserve. Recent evidences suggest that cerebrospinal fluid (CSF) biomarkers may reflect the brain pathology in AD. We inves...

journal_title：Neurobiology of disease

authors： Dumurgier J,Paquet C,Benisty S,Kiffel C,Lidy C,Mouton-Liger F,Chabriat H,Laplanche JL,Hugon J

更新日期：2010-11-01 00:00:00

abstract：:MicroRNAs (miRNA), a class of non-coding RNAs, are emerging as important modulators of neuronal development, structure and function. A connection has been established between abnormalities in miRNA expression and miRNA-mediated gene regulation and psychiatric and neurodevelopmental disorders as well as cognitive dysfu...

journal_title：Neurobiology of disease

authors： Xu B,Hsu PK,Karayiorgou M,Gogos JA

更新日期：2012-05-01 00:00:00

abstract：:Huntington's disease (HD) is a hereditary neurodegenerative disorder caused by an expansion of a CAG repeat in the huntingtin (htt) gene, which results in an aberrant form of the protein (mhtt). This leads to motor and cognitive deficits associated with corticostriatal and hippocampal alterations. The levels of STriat...

journal_title：Neurobiology of disease

authors： García-Forn M,Martínez-Torres S,García-Díaz Barriga G,Alberch J,Milà M,Azkona G,Pérez-Navarro E

更新日期：2018-12-01 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：: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：:In the present study we analyze the molecular mechanisms underlying motor neuron degeneration in familial amyotrophic lateral sclerosis (FALS). For this, we used a transgenic mouse model expressing the Cu/Zn superoxide dismutase (SOD1) gene with a Gly(86) to Arg (G86R) mutation equivalent to that found in a subset of ...

journal_title：Neurobiology of disease

authors： Dupuis L,de Tapia M,René F,Lutz-Bucher B,Gordon JW,Mercken L,Pradier L,Loeffler JP

更新日期：2000-08-01 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：:Loss-of-function mutations in the parkin-encoding PARK2 gene are a frequent cause of young-onset, autosomal recessive Parkinson's disease (PD). Parkin knockout mice have no nigro-striatal neuronal loss but exhibit abnormalities of striatal dopamine transmission and cortico-striatal synaptic function. How these predege...

journal_title：Neurobiology of disease

authors： Baaske MK,Kramer ER,Meka DP,Engler G,Engel AK,Moll CKE

更新日期：2020-04-01 00:00:00

abstract：:Seizures occur in the basal ganglia (BG) of epileptic patients and in animal models of epilepsy, but there is relatively little known about how these events are gated and/or propagated through this structure. Here, we present and characterize a model of in vitro seizure-like events (SLEs) in the striatum by applying c...

journal_title：Neurobiology of disease

authors： Yu W,Calos M,Pilitsis J,Shin DS

更新日期：2013-04-01 00:00:00

abstract：:Major depressive disorder (MDD) is one of the most prevalent major psychiatric disorders with a lifetime prevalence of 17%. Recent evidence suggests MDD is not only a brain dysfunction, but a systemic disease affecting the whole body. Central and peripheral inflammatory changes seem to be a centerpiece of MDD patholog...

journal_title：Neurobiology of disease

authors： Money KM,Olah Z,Korade Z,Garbett KA,Shelton RC,Mirnics K

更新日期：2016-05-01 00:00:00

abstract：:Glutamatergic dysfunction is strongly implicated in schizophrenia and mood disorders. GluA1 knockout (KO) mice display schizophrenia- and depression-related abnormalities. Here, we asked whether GluA1 KO show mania-related abnormalities. KO were tested for behavior in approach/avoid conflict tests, responses to repeat...

journal_title：Neurobiology of disease

authors： Fitzgerald PJ,Barkus C,Feyder M,Wiedholz LM,Chen YC,Karlsson RM,Machado-Vieira R,Graybeal C,Sharp T,Zarate C,Harvey-White J,Du J,Sprengel R,Gass P,Bannerman D,Holmes A

更新日期：2010-12-01 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：: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