abstract：:Mechanisms determining characteristic age-of-onset for neurological diseases are largely unknown. Normal brain aging associates with robust and progressive transcriptome changes ("molecular aging"), but the intersection with disease pathways is mostly uncharacterized. Here, using cross-cohort microarray analysis of fo...

journal_title：Neurobiology of disease

authors： Glorioso C,Oh S,Douillard GG,Sibille E

更新日期：2011-02-01 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：:The endoplasmic reticulum-stress response is induced in several neurodegenerative diseases and in cellular models of Huntington's disease. However, here we report that the processing of ATF6α to its active nuclear form, one of the three branches of endoplasmic reticulum-stress activation, is impaired in both animal mo...

journal_title：Neurobiology of disease

authors： Fernandez-Fernandez MR,Ferrer I,Lucas JJ

更新日期：2011-01-01 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：: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：:Huntington disease (HD) is a fatal neurodegenerative disorder caused by an expanded CAG repeat. Its length can be used to estimate the time of clinical diagnosis, which is defined by overt motor symptoms. Non-motor symptoms begin before motor onset, and involve changes in hypothalamus-regulated functions such as sleep...

journal_title：Neurobiology of disease

authors： Soneson C,Fontes M,Zhou Y,Denisov V,Paulsen JS,Kirik D,Petersén A,Huntington Study Group PREDICT-HD investigators.

更新日期：2010-12-01 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：: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：: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：:Oxidative stress is involved in the pathogenesis of various neurodegenerative disorders, conventional antioxidant strategies have yet been of limited success. We have employed transgenic Caenorhabditis elegans expressing DsRed2 in dopaminergic neurons and CFP pan-neuronally, to characterize in larval and adult animals...

journal_title：Neurobiology of disease

authors： Mocko JB,Kern A,Moosmann B,Behl C,Hajieva P

更新日期：2010-10-01 00:00:00

abstract：:Neurodegenerative diseases impose a burden on society, yet for the most part, the mechanisms underlying neuronal dysfunction and death in these disorders remain unclear despite the identification of relevant disease genes. Given the molecular conservation in neuronal signaling pathways across vertebrate and invertebra...

journal_title：Neurobiology of disease

authors： Dimitriadi M,Hart AC

更新日期：2010-10-01 00:00:00

abstract：:Neurodegenerative diseases encompass a large group of neurological disorders. Clinical symptoms can include memory loss, cognitive impairment, loss of movement or loss of control of movement, and loss of sensation. Symptoms are typically adult onset (although severe cases can occur in adolescents) and are reflective o...

journal_title：Neurobiology of disease

authors： Ambegaokar SS,Roy B,Jackson GR

更新日期：2010-10-01 00:00:00

abstract：:In amyotrophic lateral sclerosis (ALS), the exogenous temporal triggers that result in initial motor neuron death are not understood. Overactivation and consequent accelerated loss of vulnerable motor neurons is one theory of disease initiation. The vulnerability of motor neurons in response to chronic peripheral nerv...

journal_title：Neurobiology of disease

authors： Lepore AC,Tolmie C,O'Donnell J,Wright MC,Dejea C,Rauck B,Hoke A,Ignagni AR,Onders RP,Maragakis NJ

更新日期：2010-09-01 00:00:00

abstract：:Transglutaminase 2 (TG2) is a multifunctional protein that modulates cell survival and death pathways. It is upregulated in numerous ischemic models, and protects primary neurons from oxygen and glucose deprivation. TG2 binds to the hypoxia inducible factor (HIF) 1beta and decreases the upregulation of hypoxic-induced...

journal_title：Neurobiology of disease

authors： Filiano AJ,Tucholski J,Dolan PJ,Colak G,Johnson GV

更新日期：2010-09-01 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：: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：:Huntington's disease (HD) is a devastating disorder that affects approximately 1 in 10,000 people and is accompanied by neuronal dysfunction and neurodegeneration. HD manifests as a progressive chorea, a decline in mental abilities accompanied by behavioural, emotional and psychiatric problems followed by, dementia, a...

journal_title：Neurobiology of disease

authors： Buckley NJ,Johnson R,Zuccato C,Bithell A,Cattaneo E

更新日期：2010-07-01 00:00:00

abstract：:The rapidly emerging science of epigenetics and epigenomic medicine promises to reveal novel insights into the susceptibility to and the onset and progression of epileptic disorders. Epigenetic regulatory mechanisms are now implicated in orchestrating aspects of neural development (e.g., cell fate specification and ma...

journal_title：Neurobiology of disease

authors： Qureshi IA,Mehler MF

更新日期：2010-07-01 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：:Blood-brain barrier (BBB) disruption, mediated through matrix metalloproteinases (MMPs) and other mechanisms, is a critical event during ischemic stroke. Tissue plasminogen activator (tPA) is the only FDA-approved thrombolytic therapy for acute ischemic stroke, but the efficacy and safety of its therapeutic applicatio...

journal_title：Neurobiology of disease

authors： Jin R,Yang G,Li G

更新日期：2010-06-01 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：: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：:Deep brain stimulation for epilepsy has garnered attention from epileptologists due to its well-documented success in treating movement disorders and the low morbidity associated with the implantation of electrodes. Given the large proportion of patients who fail medical therapy and are not candidates for surgical ame...

journal_title：Neurobiology of disease

authors： Lega BC,Halpern CH,Jaggi JL,Baltuch GH

更新日期：2010-06-01 00:00:00

abstract：:TRIM family proteins are involved in a broad range of biological processes, and their alteration results in many diverse pathological conditions found in genetic diseases, viral infections, and cancers. However, the spatial and temporal expression and function of TRIM9, one of TRIM family proteins, remain obscure. Our...

journal_title：Neurobiology of disease

authors： Tanji K,Kamitani T,Mori F,Kakita A,Takahashi H,Wakabayashi K

更新日期：2010-05-01 00:00:00

abstract：:The hippocampus is often injured in neonatal stroke. We have investigated the effect of erythropoietin (EPO) on oxygen-glucose deprived hippocampal slices and hypoxic progenitor cells. EPO improved survival of the organotypic hippocampal slices with significantly less cell death in the dentate gyrus and an increased n...

journal_title：Neurobiology of disease

authors： Osredkar D,Sall JW,Bickler PE,Ferriero DM

更新日期：2010-05-01 00:00:00

abstract：:A crucial question in polyQ-induced neurodegeneration is the influence of wild type protein on the formation of aggregates and toxicity. Recently it was shown that non-expanded ataxin-3 protein mitigated neurodegeneration in a Drosophila and mouse model of SCA3. We now explored the effects of overexpressing non-expand...

journal_title：Neurobiology of disease

authors： Hübener J,Riess O

更新日期：2010-04-01 00:00:00

abstract：:Lyme disease, caused by the bacterium Borrelia burgdorferi, can cause multi-systemic signs and symptoms, including peripheral and central nervous system disease. This review examines the evidence for and mechanisms of inflammation in neurologic Lyme disease, with a specific focus on the central nervous system, drawing...

journal_title：Neurobiology of disease

authors： Fallon BA,Levin ES,Schweitzer PJ,Hardesty D

更新日期：2010-03-01 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：:Whether seizures in the developing brain cause long-term changes in the mature brain has been debated. We tested the hypothesis that a model of early-life seizures, induced by systemic injection of a GABA(B) receptor antagonist CGP56999A in immature rats, decreased GABA(B) receptor-mediated inhibitory postsynaptic cur...

journal_title：Neurobiology of disease

authors： Qu L,Boyce R,Leung LS

更新日期：2010-03-01 00:00:00

abstract：:The mechanism of status epilepticus-induced neuronal death in the immature brain is not fully understood. In the present study, we examined the contribution of caspases in our lithium-pilocarpine model of status epilepticus in 14 days old rat pups. In CA1, upregulation of caspase-8, but not caspase-9, preceded caspase...

journal_title：Neurobiology of disease

authors： Lopez-Meraz ML,Wasterlain CG,Rocha LL,Allen S,Niquet J

更新日期：2010-02-01 00:00:00

abstract：:Excitotoxicity plays a major role in the pathogenesis of Huntington disease (HD), a fatal neurodegenerative disorder. Adenosine A(2A) receptors (A(2A)Rs) modulate excitotoxicity and have been suggested to play a pathogenetic role in HD. The main aim of this study was to evaluate the effect of A(2A)R blockade on the ex...

journal_title：Neurobiology of disease

authors： Martire A,Ferrante A,Potenza RL,Armida M,Ferretti R,Pézzola A,Domenici MR,Popoli P

更新日期：2010-01-01 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 central nervous system is protected by barriers which control the entry of compounds into the brain, thereby regulating brain homeostasis. The blood-brain barrier, formed by the endothelial cells of the brain capillaries, restricts access to brain cells of blood-borne compounds and facilitates nutrients essential ...

journal_title：Neurobiology of disease

authors： Gabathuler R

更新日期：2010-01-01 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：:Both the cellular prion protein (PrP(c)) and the amyloid precursor protein (APP) are physiologically subjected to complex proteolytic processing events. While for APP the proteinases involved--alpha-, beta- and gamma-secretase--have been identified in vitro and in vivo, the cleavage of PrP(c) by now has been linked on...

journal_title：Neurobiology of disease

authors： Endres K,Mitteregger G,Kojro E,Kretzschmar H,Fahrenholz F

更新日期：2009-11-01 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：:There is no information concerning signal transduction mechanisms downstream of the opioid/nociceptin receptors in the human epileptic brain. The aim of this work was to evaluate the level of G-proteins activation mediated by DAMGO (a mu receptor selective peptide) and nociceptin, and the binding to mu and nociceptin ...

journal_title：Neurobiology of disease

authors： Rocha L,Orozco-Suarez S,Alonso-Vanegas M,Villeda-Hernandez J,Gaona A,Páldy E,Benyhe S,Borsodi A

更新日期：2009-09-01 00:00:00

abstract：:3-Nitropropionic acid (3-NP) is an irreversible inhibitor of succinate dehydrogenase that has been used to explore the primary mechanisms of cell death associated with mitochondrial dysfunction and neurodegeneration in Huntington's disease. In this study we investigated the ability of brain-derived neurotrophic factor...

journal_title：Neurobiology of disease

authors： Almeida S,Laço M,Cunha-Oliveira T,Oliveira CR,Rego AC

更新日期：2009-09-01 00:00:00

abstract：:The repertoire of biochemicals (or small molecules) present in cells, tissue, and body fluids is known as the metabolome. Today, clinicians utilize only a very small part of the information contained in the metabolome, as revealed by the quantification of a limited set of analytes to gain information on human health. ...

journal_title：Neurobiology of disease

authors： Quinones MP,Kaddurah-Daouk R

更新日期：2009-08-01 00:00:00

abstract：:Butyrylcholinesterase (BChE) genotypes and protein (BuChE) activity, especially in combination with Apolipoprotein E4 (ApoE4), have been investigated as risk factors for developing Alzheimer disease (AD) and may be associated with the rate of progression of cognitive decline. Despite similar pathologic (e.g. amyloid d...

journal_title：Neurobiology of disease

authors： Maetzler W,Keller S,Michelis J,Koehler N,Stransky E,Becker C,Schulte C,Melms A,Gasser T,Berg D

更新日期：2009-08-01 00:00:00