Transgenic expression of an expanded (GCG)13 repeat PABPN1 leads to weakness and coordination defects in mice.

abstract：:In a transgenic mouse model of the neurodegenerative disorder Huntington's disease (HD), age-dependent neurologic defects are accompanied by progressive alterations in glucose tolerance that culminate in the development of diabetes mellitus and insulin deficiency. Pancreatic islets from HD transgenic mice express redu...

journal_title：Neurobiology of disease

authors： Andreassen OA,Dedeoglu A,Stanojevic V,Hughes DB,Browne SE,Leech CA,Ferrante RJ,Habener JF,Beal MF,Thomas MK

更新日期：2002-12-01 00:00:00

abstract：:Several neurodegenerative diseases like Huntington's, a polyglutamine (PolyQ) disease, are initiated by protein aggregation in neurons. Furthermore, these diseases are also associated with a multitude of responses in non-neuronal cells in the brain, in particular glial cells, like astrocytes. These non-neuronal respon...

journal_title：Neurobiology of disease

authors： Bason M,Meister-Broekema M,Alberts N,Dijkers P,Bergink S,Sibon OCM,Kampinga HH

更新日期：2019-04-01 00:00:00

abstract：:Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by motor neuron death. A 20% of familial ALS cases are associated with mutations in the gene coding for superoxide dismutase 1 (SOD1). The accumulation of abnormal aggregates of different proteins is a common feature in motor neurons of p...

journal_title：Neurobiology of disease

authors： Pinto C,Medinas DB,Fuentes-Villalobos F,Maripillán J,Castro AF,Martínez AD,Osses N,Hetz C,Henríquez JP

更新日期：2019-10-01 00:00:00

abstract：:G-protein coupled receptor 3 (GPR3), GPR6, and GPR12 belong to a family of constitutively active Gs-coupled receptors that activate 3'-5'-cyclic adenosine monophosphate (cAMP) and are highly expressed in the brain. Among these receptors, the endogenous expression of GPR3 in cerebellar granule neurons (CGNs) is increas...

journal_title：Neurobiology of disease

authors： Tanaka S,Miyagi T,Dohi E,Seki T,Hide I,Sotomaru Y,Saeki Y,Antonio Chiocca E,Matsumoto M,Sakai N

更新日期：2014-08-01 00:00:00

abstract：:The amyloid precursor protein (APP) is a type I transmembrane protein translocated to neuronal terminals, whose function is still unknown. The C-terminus of APP mediates its interaction with cellular adaptor and signaling proteins, some of which signal to the stress-activated protein kinase (SAPK) pathway. Here we sho...

journal_title：Neurobiology of disease

authors： Galvan V,Banwait S,Spilman P,Gorostiza OF,Peel A,Ataie M,Crippen D,Huang W,Sidhu G,Ichijo H,Bredesen DE

更新日期：2007-10-01 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：:Huntington's disease (HD) causes severe motor impairments that are characterized by chorea, dystonia, and impaired fine motor control. The motor deficits include deficits in the control of the forelimb, but as yet there has been no comprehensive assessment of the impairments in arm, hand and digit movements as they ar...

journal_title：Neurobiology of disease

authors： Klein A,Sacrey LA,Dunnett SB,Whishaw IQ,Nikkhah G

更新日期：2011-02-01 00:00:00

abstract：:Beta-amyloid (Abeta) deposition is one important pathological hallmark in Alzheimer's disease (AD). However, low levels of Abeta may modify critical endogenous protection systems before neurodegeneration occurs. We examined the time-course effect of sublethal concentrations of Abeta on total BDNF (panBDNF), BDNF trans...

journal_title：Neurobiology of disease

authors： Aliaga E,Silhol M,Bonneau N,Maurice T,Arancibia S,Tapia-Arancibia L

更新日期：2010-01-01 00:00:00

abstract：:The brain of Alzheimer's disease patients shows abundant dystrophic neurites in close proximity to fibrillar beta-amyloid (A beta) plaques, and activated glial cells. We evaluated the influence of pro-inflammatory molecules (LPS + IFN-gamma) on A beta(1-42) neurotoxicity. 2 microM A beta(1-42) induced apoptosis of hip...

journal_title：Neurobiology of disease

authors： Ramírez G,Toro R,Döbeli H,von Bernhardi R

更新日期：2005-06-01 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：:To decipher the pathway of apoptosis induction downstream to caspase-8 activation by exogenous expression of Hippi, an interactor of huntingtin-interacting protein Hip1, we studied apoptosis in HeLa and Neuro2A cells expressing GFP-tagged Hippi. Nuclear fragmentation, caspase-1, caspase-8, caspase-9/caspase-6 and casp...

journal_title：Neurobiology of disease

authors： Majumder P,Chattopadhyay B,Mazumder A,Das P,Bhattacharyya NP

更新日期：2006-05-01 00:00:00

abstract：:Deletion of the mTOR pathway inhibitor PTEN from postnatally-generated hippocampal dentate granule cells causes epilepsy. Here, we conducted field potential, whole cell recording and single cell morphology studies to begin to elucidate the mechanisms by which granule cell-specific PTEN-loss produces disease. Cells fro...

journal_title：Neurobiology of disease

authors： Santos VR,Pun RYK,Arafa SR,LaSarge CL,Rowley S,Khademi S,Bouley T,Holland KD,Garcia-Cairasco N,Danzer SC

更新日期：2017-12-01 00:00:00

abstract：:Cytokine-dependent mechanisms in the CNS have been implicated in the pathogenesis of depression. Interleukin-6 is upregulated in depressed patients and dowregulated by antidepressants. It is, however, unknown whether IL-6 is involved in the pathogenesis of depression. We subjected IL-6-deficient mice (IL-6(-/-)) to de...

journal_title：Neurobiology of disease

authors： Chourbaji S,Urani A,Inta I,Sanchis-Segura C,Brandwein C,Zink M,Schwaninger M,Gass P

更新日期：2006-09-01 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：: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：:Clinical studies indicate that phenytoin prevents acute post-traumatic seizures but not subsequent post-traumatic epilepsy. We explored this phenomenon using organotypic hippocampal slice cultures as a model of severe traumatic brain injury. Hippocampal slices were cultured for up to eight weeks, during which acute an...

journal_title：Neurobiology of disease

authors： Berdichevsky Y,Dzhala V,Mail M,Staley KJ

更新日期：2012-02-01 00:00:00

abstract：:Clinical and experimental evidence point to a possible role of cerebrovascular dysfunction in Alzheimer's disease (AD). The 5xFAD mouse model of AD expresses human amyloid precursor protein and presenilin genes with mutations found in AD patients. It remains unknown whether amyloid deposition driven by these mutations...

journal_title：Neurobiology of disease

authors： Giannoni P,Arango-Lievano M,Neves ID,Rousset MC,Baranger K,Rivera S,Jeanneteau F,Claeysen S,Marchi N

更新日期：2016-04-01 00:00:00

abstract：:Traumatic brain injury (TBI) leads to acute necrosis at the site of injury followed by a sequence of secondary events lasting from hours to weeks and often years. Targeting mitochondrial impairment following TBI has shown improvements in brain mitochondrial bioenergetics and neuronal function. Recently formoterol, a h...

journal_title：Neurobiology of disease

authors： Vekaria HJ,Hubbard WB,Scholpa NE,Spry ML,Gooch JL,Prince SJ,Schnellmann RG,Sullivan PG

更新日期：2020-07-01 00:00:00

abstract：:Parkinson's disease (PD) is a neurodegenerative disorder caused by loss of dopaminergic neurons. Although many reports have suggested that genetic factors are implicated in the pathogenesis of PD, molecular mechanisms underlying selective dopaminergic neuronal degeneration remain unknown. DJ-1 is a causative gene for ...

journal_title：Neurobiology of disease

authors： Usami Y,Hatano T,Imai S,Kubo S,Sato S,Saiki S,Fujioka Y,Ohba Y,Sato F,Funayama M,Eguchi H,Shiba K,Ariga H,Shen J,Hattori N

更新日期：2011-09-01 00:00:00

abstract：:Parkinson's Disease (PD) and Multiple System Atrophy (MSA) are neurodegenerative diseases characterized neuropathologically by alpha-synuclein accumulation in brain cells. This accumulation is hypothesized to contribute to constitutive neuroinflammation, and to participate in the neurodegeneration. Cytokines, which ar...

journal_title：Neurobiology of disease

authors： Rydbirk R,Elfving B,Andersen MD,Langbøl MA,Folke J,Winge K,Pakkenberg B,Brudek T,Aznar S

更新日期：2017-10-01 00:00:00

abstract：:Tamoxifen (TAM) is a selective estrogen receptor modulator, widely used in the treatment and prevention of estrogen-dependent breast cancer. Although with great clinical results, women on TAM therapy still report several side effects, such as sexual dysfunction, which impairs quality of life. The anatomo-functional su...

journal_title：Neurobiology of disease

authors： Sá SI,Teixeira N,Fonseca BM

更新日期：2018-01-01 00:00:00

abstract：:Males and females show a different predisposition to certain types of seizures in clinical studies. Animal studies have provided growing evidence for sexual dimorphism of certain brain regions, including those that control seizures. Seizures are modulated by networks involving subcortical structures, including thalamu...

journal_title：Neurobiology of disease

authors： Giorgi FS,Galanopoulou AS,Moshé SL

更新日期：2014-12-01 00:00:00

abstract：:Environmental stimulation throughout development adjusts the neurobehavioral systems involved in learning, memory and defensive responses. Environment-mediated phenotypic plasticity can be considered from two different, yet complementary, viewpoints. On one hand, the possibility that environmental interventions protec...

journal_title：Neurobiology of disease

authors： Laviola G,Hannan AJ,Macrì S,Solinas M,Jaber M

更新日期：2008-08-01 00:00:00

abstract：:Background While significant advances have been made in uncovering the aetiology of Alzheimer's disease and related dementias at the genetic level, molecular events at the epigenetic level remain largely undefined. Emerging evidence indicates that small non-coding RNAs (sncRNAs) and their associated RNA modifications ...

journal_title：Neurobiology of disease

authors： Zhang X,Trebak F,Souza LAC,Shi J,Zhou T,Kehoe PG,Chen Q,Feng Earley Y

更新日期：2020-11-01 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：:Alpha-synuclein containing cellular inclusions are a hallmark of Parkinson Disease, Lewy Body Dementia, and Multiple System Atrophy. A genome wide expression screen was performed in C. elegans overexpressing both wild-type and A53T human alpha-synuclein. 433 genes were up- and 67 genes down-regulated by statistical an...

journal_title：Neurobiology of disease

authors： Vartiainen S,Pehkonen P,Lakso M,Nass R,Wong G

更新日期：2006-06-01 00:00:00

abstract：:Huntington's disease (HD) is a genetic neurodegenerative disease characterized by movement disorders, cognitive decline and neuropsychiatric symptoms. HD is caused by expanded CAG tract within the coding region of Huntingtin protein. Despite major insights into the molecular mechanisms leading to HD, no effective cure...

journal_title：Neurobiology of disease

authors： Dupont P,Besson MT,Devaux J,Liévens JC

更新日期：2012-08-01 00:00:00

abstract：:Microglia, a type of immune cell in the brain, are in a ramified status with branched processes in normal conditions. Upon pathological stimulation, microglia retract their processes and become activated. Searching methods to make the activated microglia return to ramified status would help cope with injuries induced ...

journal_title：Neurobiology of disease

authors： Wang P,Zhang Y,Gong Y,Yang R,Chen Z,Hu W,Wu Y,Gao M,Xu X,Qin Y,Huang C

更新日期：2018-03-01 00:00:00

abstract：:To identify potential multiple sclerosis (MS)-specific biomarkers, we used a proteomic approach to screen cerebrospinal fluid (CSF) from 40 MS patients and 13 controls. We identified seven proteins (Beta-2-microglobulin, Bri2-23, Fetuin-A, Kallikrein-6, Plasminogen, Ribonuclease-1, and Transferrin) that had significan...

journal_title：Neurobiology of disease

authors： Harris VK,Diamanduros A,Good P,Zakin E,Chalivendra V,Sadiq SA

更新日期：2010-10-01 00:00:00

abstract：:Evidence of the gut microbiota influencing neurodegenerative diseases has been reported for several neural diseases. However, there is little insight regarding the relationship between the gut microbiota and prion disease. Here, using fecal samples of 12 prion-infected mice and 25 healthy controls, we analyzed the str...

journal_title：Neurobiology of disease

authors： Yang D,Zhao D,Shah SZA,Wu W,Lai M,Zhang X,Li J,Guan Z,Zhao H,Li W,Gao H,Zhou X,Yang L

更新日期：2020-02-01 00:00:00