Reducing canonical Wingless/Wnt signaling pathway confers protection against mutant Huntingtin toxicity in Drosophila.

abstract：:Toll-like receptors (TLRs) are mediators of the innate immune response to exogenous pathogens. They have also been implicated in sterile inflammation associated with systemic injury and non-infectious diseases via binding of endogenous ligands, possibly released by damaged cells. Emerging evidence indicates that some ...

journal_title：Neurobiology of disease

authors： David BT,Ratnayake A,Amarante MA,Reddy NP,Dong W,Sampath S,Heary RF,Elkabes S

更新日期：2013-06-01 00:00:00

abstract：:Pathological gambling (PG) represents a behavioral side effect of dopamine replacement therapy in a minority of patients with Parkinson's disease (PD). Using striatal dopamine transporter (DAT) with single photon emission tomography we assessed presynaptic dopaminergic function in 8 PD patients with PG, 21 matched PD ...

journal_title：Neurobiology of disease

authors： Cilia R,Ko JH,Cho SS,van Eimeren T,Marotta G,Pellecchia G,Pezzoli G,Antonini A,Strafella AP

更新日期：2010-07-01 00:00:00

abstract：:Hereditary sensory neuropathy type 1 (HSN-1) is a peripheral neuropathy most frequently caused by mutations in the SPTLC1 or SPTLC2 genes, which code for two subunits of the enzyme serine palmitoyltransferase (SPT). SPT catalyzes the first step of de novo sphingolipid synthesis. Mutations in SPT result in a change in ...

journal_title：Neurobiology of disease

authors： Wilson ER,Kugathasan U,Abramov AY,Clark AJ,Bennett DLH,Reilly MM,Greensmith L,Kalmar B

更新日期：2018-09-01 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：:NMDA receptor dysfunction is central to the encephalopathies caused by missense mutations in the NMDA receptor subunit genes. Missense variants of GRIN1, GRIN2A, and GRIN2B cause similar syndromes with varying severity of intellectual impairment, autism, epilepsy, and motor dysfunction. To gain insight into possible b...

journal_title：Neurobiology of disease

authors： Intson K,van Eede MC,Islam R,Milenkovic M,Yan Y,Salahpour A,Henkelman RM,Ramsey AJ

更新日期：2019-12-01 00:00:00

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：: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：: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：BACKGROUND/INTRODUCTION:The neuroinflammatory response plays a major role in the secondary injury cascade after traumatic spinal cord injury (SCI). To date, systemic anti-inflammatory medications such as methylprednisolone sodium succinate (MPSS) have shown promise in SCI. However, systemic immunosuppression can have d...

journal_title：Neurobiology of disease

authors： Chio JCT,Wang J,Surendran V,Li L,Zavvarian MM,Pieczonka K,Fehlings MG

更新日期：2021-01-01 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：:Charcot-Marie-Tooth disease is a commonly inherited form of neuropathy. Although named over 100 years ago, identification of subtypes of Charcot-Marie-Tooth has rapidly expanded in the preceding decades with the advancement of genetic sequencing, including type 2F (CMT2F), due to mutations in heat shock protein 27 (Hs...

journal_title：Neurobiology of disease

authors： Schwartz NU

更新日期：2019-10-01 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：: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：: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：:Infection of non-neuronal cell types with herpes simplex virus type 1 (HSV-1) results in the degradation of host mRNA (Kwong & Frenkel 1987) and a shutoff in host protein synthesis (Roizman et al. 1965). This effect is mediated by a virion associated protein that is encoded by the viral vhs gene (Read & Frenkel 1983)....

journal_title：Neurobiology of disease

authors： Nichol PF,Chang JY,Johnson EM Jr,Olivo PD

更新日期：1994-11-01 00:00:00

abstract：:Chronic inflammation is known to play an important role in the heterogeneous pathogenesis of Alzheimer's disease (AD). Activated astrocytes expressing glial fibrillary acidic protein (GFAP) are closely associated with AD pathology, such as tangles, neuritic plaques and amyloid depositions. Altogether, 46 soluble isofo...

journal_title：Neurobiology of disease

authors： Korolainen MA,Auriola S,Nyman TA,Alafuzoff I,Pirttilä T

更新日期：2005-12-01 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：:Intracerebroventricular administration of kainic acid in the adult rat, a widely used model for studying human temporal lobe epilepsy, results in widespread degeneration of CA3-pyramidal neurons. Transplantation of specific fetal hippocampal CA3 cell grafts into the lesioned CA3-region at a prolonged post lesion delay...

journal_title：Neurobiology of disease

authors： Zaman V,Shetty AK

更新日期：2001-12-01 00:00:00

abstract：:The terms "neuroacanthocytosis" (NA) and "neurodegeneration with brain iron accumulation" (NBIA) both refer to groups of genetically heterogeneous disorders, classified together due to similarities of their phenotypic or pathological findings. Even collectively, the disorders that comprise these sets are exceedingly r...

journal_title：Neurobiology of disease

authors： Prohaska R,Sibon OC,Rudnicki DD,Danek A,Hayflick SJ,Verhaag EM,Vonk JJ,Margolis RL,Walker RH

更新日期：2012-06-01 00:00:00

abstract：:Metabotropic glutamate receptor 5 (mGluR5) is a drug target for central nervous system disorders such as fragile X syndrome that involve excessive glutamate-induced excitation. We tested the efficacy of a novel negative allosteric modulator of mGluR5 developed by Merz Pharmaceuticals, MRZ-8456, in comparison to MPEP a...

journal_title：Neurobiology of disease

authors： Westmark PR,Dekundy A,Gravius A,Danysz W,Westmark CJ

更新日期：2018-11-01 00:00:00

abstract：:Using conventional in vitro extracellular field potential recordings we have investigated both short- and long-term synaptic plasticity in the hippocampal CA1 area of mice infected with ME7 scrapie. In agreement with earlier studies, no changes were seen in the properties of the Schäffer collateralevoked field excitat...

journal_title：Neurobiology of disease

authors： Johnston AR,Fraser JR,Jeffrey M,MacLeod N

更新日期：1998-09-01 00:00:00

abstract：:Focal mechanical cortical trauma triggers diffuse apoptotic neurodegeneration in the developing rat brain which is associated with invasion of brain tissue with inflammatory mediators. We hypothesized that caspase-1 and the two caspase-1-processed cytokines, interleukin (IL)-1beta and IL-18, are involved in trauma-ind...

journal_title：Neurobiology of disease

authors： Sifringer M,Stefovska V,Endesfelder S,Stahel PF,Genz K,Dzietko M,Ikonomidou C,Felderhoff-Mueser U

更新日期：2007-03-01 00:00:00

abstract：:Recent work has demonstrated the importance of miRNAs in the pathogenesis of various brain disorders including the neurodegenerative disorder spinocerebellar ataxia (SCA). This review focuses on the role of miRNAs in the shared pathogenesis of the different SCA types. We examine the novel findings of a recent cell-typ...

journal_title：Neurobiology of disease

authors： van der Stijl R,Withoff S,Verbeek DS

更新日期：2017-12-01 00:00:00

abstract：:Rett syndrome (RS) is caused by mutations in the gene encoding methyl-CpG-binding protein 2 (MeCP2) and is characterized by arrested postnatal neurodevelopment. We followed the expression of MeCP2 protein in various brain structures of normal rat from birth to 2 years of age. By measuring the amount of protein using t...

journal_title：Neurobiology of disease

authors： Cassel S,Revel MO,Kelche C,Zwiller J

更新日期：2004-03-01 00:00:00

abstract：:It is well known that the uncoupling between local cerebral glucose utilization (LCGU) and local cerebral blood flow (LCBF), i.e. decrease in LCBF rates with high LCGU, is frequently associated with seizure-induced neuronal damage. This study was performed to assess if the neuroprotective effect of the adenosinergic A...

journal_title：Neurobiology of disease

authors： Silva IR,Nehlig A,Rosim FE,Vignoli T,Persike DS,Ferrandon A,Sinigaglia-Coimbra R,Fernandes MJ

更新日期：2011-01-01 00:00:00

abstract：:Posttraumatic epilepsy (PTE) is a major neurodegenerative disease accounting for 20% of symptomatic epilepsy cases. A long latent phase offers a potential window for prophylactic treatment strategies to prevent epilepsy onset, provided that the patients at risk can be identified. Some promising imaging biomarker candi...

journal_title：Neurobiology of disease

authors： Immonen R,Harris NG,Wright D,Johnston L,Manninen E,Smith G,Paydar A,Branch C,Grohn O

更新日期：2019-03-01 00:00:00

abstract：:Glutathione (GSH) is an important neuroprotective molecule in the brain. The strategy to increase neuronal GSH level is a promising approach to the treatment of neurodegenerative diseases. However, the regulatory mechanism by which neuron-specific GSH synthesis is facilitated remains elusive. Glutamate transporter-ass...

journal_title：Neurobiology of disease

authors： Aoyama K,Wang F,Matsumura N,Kiyonari H,Shioi G,Tanaka K,Kinoshita C,Kikuchi-Utsumi K,Watabe M,Nakaki T

更新日期：2012-03-01 00:00:00

abstract：:Huntington's disease (HD) is a lethal, neurodegenerative disorder caused by expansion of the polyglutamine repeat in the Huntingtin gene (HTT), leading to mutant protein misfolding, aggregation, and neuronal death. Feeding a Drosophila HD model cystamine, or expressing a transgene encoding the anti-htt intracellular a...

journal_title：Neurobiology of disease

authors： Bortvedt SF,McLear JA,Messer A,Ahern-Rindell AJ,Wolfgang WJ

更新日期：2010-10-01 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：:Depression affects at least 322 million people globally, or approximately 4.4% of the world's population. While the earnestness of researchers and clinicians to understand and treat depression is not waning, the number of individuals suffering from depression continues to increase over and above the rate of global pop...

journal_title：Neurobiology of disease

authors： Flux MC,Lowry CA

更新日期：2020-02-01 00:00:00