Purkinje cell-specific Grip1/2 knockout mice show increased repetitive self-grooming and enhanced mGluR5 signaling in cerebellum.

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：: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：:The dysregulation of glycogen synthase kinase-3 (GSK3) has been implicated in Alzheimer disease (AD) pathogenesis and in Abeta-induced neurotoxicity, leading us to investigate it as a therapeutic target in an intracerebroventricular Abeta infusion model. Infusion of a specific GSK3 inhibitor SB216763 (SB) reduced a do...

journal_title：Neurobiology of disease

authors： Hu S,Begum AN,Jones MR,Oh MS,Beech WK,Beech BH,Yang F,Chen P,Ubeda OJ,Kim PC,Davies P,Ma Q,Cole GM,Frautschy SA

更新日期：2009-02-01 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：:Synaptic neurodegeneration is thought to be an early event initiated by soluble β-amyloid (Aβ) aggregates that closely correlates with cognitive decline in Alzheimer disease (AD). Apolipoprotein ε4 (APOE4) is the most common genetic risk factor for both familial AD (FAD) and sporadic AD; it accelerates Aβ aggregation ...

journal_title：Neurobiology of disease

authors： Ma QL,Teng E,Zuo X,Jones M,Teter B,Zhao EY,Zhu C,Bilousova T,Gylys KH,Apostolova LG,LaDu MJ,Hossain MA,Frautschy SA,Cole GM

更新日期：2018-06-01 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：:Giliosis is a hallmark of prion disease. A neurotoxic prion peptide (PrP106-126) induces astrocyte proliferation in the presence of microglia. This peptide also directly enhances microglial proliferation in culture. We have investigated this further to understand the method by which factors released by microglia and P...

journal_title：Neurobiology of disease

authors： Brown DR,Schmidt B,Kretzschmar HA

更新日期：1998-04-01 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：:Amyotrophic lateral sclerosis (ALS) is a chronic neurodegenerative disease characterized by the progressive loss of motor neurons, leading to profound weakness and eventual death of affected individuals. For the vast majority of patients with ALS, the etiology of the disorder is unknown, and although multiple clinical...

journal_title：Neurobiology of disease

authors： Elliott JL

更新日期：1999-10-01 00:00:00

abstract：:Although the incidence of seizures after a cerebrovascular event including intracerebral hemorrhage has been widely recognized, the present studies have demonstrated that generalized convulsive seizures can cause multifocal amygdaloallocortical hemorrhage and tissue necrosis, the origin of which remains to be establis...

journal_title：Neurobiology of disease

authors： Mraovitch S,Calando Y,Régnier A,Lamproglou I,Vicaut E

更新日期：2005-08-01 00:00:00

abstract：:Rare de novo mutations in genes associated with inherited Mendelian disorders are potential contributors to sporadic disease. DYT1 dystonia is an autosomal dominant, early-onset, generalized dystonia associated with an in-frame, trinucleotide deletion (n. delGAG, p. ΔE 302/303) in the Tor1a gene. Here we examine the s...

journal_title：Neurobiology of disease

authors： Bhagat SL,Qiu S,Caffall ZF,Wan Y,Pan Y,Rodriguiz RM,Wetsel WC,Badea A,Hochgeschwender U,Calakos N

更新日期：2016-09-01 00:00:00

abstract：:Huntington's disease (HD) is one of the few neurodegenerative diseases with a known genetic cause, knowledge that has enabled the creation of animal models using genetic manipulations that aim to recapitulate HD pathology. The study of behavioral and neuropathological phenotypes of these HD models, however, has been p...

journal_title：Neurobiology of disease

authors： Menalled L,El-Khodor BF,Patry M,Suárez-Fariñas M,Orenstein SJ,Zahasky B,Leahy C,Wheeler V,Yang XW,MacDonald M,Morton AJ,Bates G,Leeds J,Park L,Howland D,Signer E,Tobin A,Brunner D

更新日期：2009-09-01 00:00:00

abstract：:alpha-Synuclein has been identified as a major component of Lewy body inclusions, which are one of the pathologic hallmarks of idiopathic Parkinson's disease. Mutations in alpha-synuclein have been found to be responsible for rare familial cases of Parkinsonism. To test whether overexpression of human alpha-synuclein ...

journal_title：Neurobiology of disease

authors： Matsuoka Y,Vila M,Lincoln S,McCormack A,Picciano M,LaFrancois J,Yu X,Dickson D,Langston WJ,McGowan E,Farrer M,Hardy J,Duff K,Przedborski S,Di Monte DA

更新日期：2001-06-01 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：:Reelin is a glycoprotein that modulates synaptic function and plasticity in the mature brain, thereby favouring memory formation. We recently reported altered cerebral Reelin expression in Alzheimer's disease (AD). Here we demonstrate pronounced Reelin changes at protein and mRNA levels in the frontal cortex in adult ...

journal_title：Neurobiology of disease

authors： Botella-López A,Cuchillo-Ibáñez I,Cotrufo T,Mok SS,Li QX,Barquero MS,Dierssen M,Soriano E,Sáez-Valero J

更新日期：2010-03-01 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：:Infantile neuronal ceroid lipofuscinosis (INCL) is a severe neurodegenerative disorder of children, characterized by selective death of neocortical neurons. To understand early disease mechanisms in INCL, we have studied Ppt1(Deltaex4) knock-out mouse neurons in culture and acute brain slices. Global transcript profil...

journal_title：Neurobiology of disease

authors： Ahtiainen L,Kolikova J,Mutka AL,Luiro K,Gentile M,Ikonen E,Khiroug L,Jalanko A,Kopra O

更新日期：2007-10-01 00:00:00

abstract：:Immunization of mouse models of Alzheimer disease (AD) with amyloid-peptide (Abeta) reduces Abeta deposits and attenuates their memory and learning deficits. Recent clinical trials were halted due to meningoencephalitis, presumably induced by T cell mediated and/or Fc-mediated immune responses. Because injection of an...

journal_title：Neurobiology of disease

authors： Fukuchi K,Tahara K,Kim HD,Maxwell JA,Lewis TL,Accavitti-Loper MA,Kim H,Ponnazhagan S,Lalonde R

更新日期：2006-09-01 00:00:00

abstract：:The interplay between excitatory and inhibitory circuits underlies the brain's processes and their dysregulation has been linked to cognitive decline, psychiatric disorders and epilepsy. In patients with Alzheimer's disease (AD), an elevated occurrence of seizures has been observed in both sporadic and familial forms ...

journal_title：Neurobiology of disease

authors： Vico Varela E,Etter G,Williams S

更新日期：2019-07-01 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：:Certain clinical features of schizophrenia, such as working memory disturbances, appear to emerge from altered gamma oscillatory activity in the prefrontal cortex (PFC). Given the essential role of GABA neurotransmission in both working memory and gamma oscillations, understanding the cellular substrate for their dist...

journal_title：Neurobiology of disease

authors： Dienel SJ,Lewis DA

更新日期：2019-11-01 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：:Parkinson's disease (PD), characterized by the loss of dopaminergic nigrostriatal projections, is a debilitating neurodegenerative disease which produces bradykinesia, rigidity, tremor and postural instability. The dopamine precursor levodopa (L-Dopa) is the most effective treatment for the amelioration of PD signs an...

journal_title：Neurobiology of disease

authors： Prescott IA,Liu LD,Dostrovsky JO,Hodaie M,Lozano AM,Hutchison WD

更新日期：2014-11-01 00:00:00

abstract：:Angelman syndrome (AS) is a neurogenetic disorder caused by loss of maternal UBE3A expression or mutation-induced dysfunction of its protein product, the E3 ubiquitin-protein ligase, UBE3A. In humans and rodents, UBE3A/Ube3a transcript is maternally imprinted in several brain regions, but the distribution of native UB...

journal_title：Neurobiology of disease

authors： Gustin RM,Bichell TJ,Bubser M,Daily J,Filonova I,Mrelashvili D,Deutch AY,Colbran RJ,Weeber EJ,Haas KF

更新日期：2010-09-01 00:00:00

abstract：:Huntington's disease (HD) is an inherited neurodegenerative disorder characterized by dyskinesia, cognitive impairment and emotional disturbances, presenting progressive neurodegeneration in the striatum and intracellular mutant Huntingtin (mHTT) aggregates in various areas of the brain. Recombinant Adeno Associated V...

journal_title：Neurobiology of disease

authors： Ceccarelli I,Fiengo P,Remelli R,Miragliotta V,Rossini L,Biotti I,Cappelli A,Petricca L,La Rosa S,Caricasole A,Pollio G,Scali C

更新日期：2016-02-01 00:00:00

abstract：:The SMN2 transgenic mouse, Tg(SMN2)89Ahmb, has emerged as the most widely used in spinal muscular atrophy (SMA) research. Here we clone the genomic integration site of the transgene and demonstrate it to be in intron 4 of the metabotropic glutamate receptor 7 (mGluR7) gene. We found that the integration of this transg...

journal_title：Neurobiology of disease

authors： Gogliotti RG,Lutz C,Jorgensen M,Huebsch K,Koh S,Didonato CJ

更新日期：2011-07-01 00:00:00

abstract：:Necroptosis is a regulated form of necrosis that is mediated by a variety of proteins including tumor necrosis factor-α (TNF-α) and receptor-interacting proteins (RIPs). TNF-α, a critical inflammatory molecule, is one of the initiating signals in the necroptosis pathway, and RIP3 acts as a switch that commits the cell...

journal_title：Neurobiology of disease

authors： Yuan S,Yu Z,Zhang Z,Zhang J,Zhang P,Li X,Li H,Shen H,Chen G

更新日期：2019-09-01 00:00:00

abstract：:The developing rodent brain is vulnerable to pharmacological blockade of N-methyl-d-aspartate (NMDA) receptors which can lead to severe and disseminated apoptotic neurodegeneration. Here, we show that systemic administration of the NMDA receptor antagonist MK801 to 7-day-old rats leads to impaired activity of extracel...

journal_title：Neurobiology of disease

authors： Hansen HH,Briem T,Dzietko M,Sifringer M,Voss A,Rzeski W,Zdzisinska B,Thor F,Heumann R,Stepulak A,Bittigau P,Ikonomidou C

更新日期：2004-07-01 00:00:00

abstract：:This article reviews three concepts related to implantable brain computer interface (BCI) devices being designed for human use: neural signal extraction primarily for motor commands, signal insertion to restore sensation, and technological challenges that remain. A significant body of literature has occurred over the ...

journal_title：Neurobiology of disease

authors： Konrad P,Shanks T

更新日期：2010-06-01 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