abstract：:Several reports indicate that the activity of the hypothalamic–pituitary–adrenal axis (HPA) is increased after a brain insult and that its down-regulation can improve detrimental outcomes associated with ischemic brain injuries.Granulocyte-colony stimulating factor (G-CSF) is a neuroprotective drug shown in the naïve ...

journal_title：Neurobiology of disease

authors： Charles MS,Ostrowski RP,Manaenko A,Duris K,Zhang JH,Tang J

更新日期：2012-07-01 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：:Expansion of polyglutamine repeats is the cause of at least nine inherited human neurodegenerative disorders, including Huntington's disease (HD). It is widely accepted that deregulation of the transcriptional coactivator CBP by expanded huntingtin (htt) plays an important role in HD molecular pathogenesis. In this st...

journal_title：Neurobiology of disease

authors： Cong SY,Pepers BA,Zhou TT,Kerkdijk H,Roos RA,van Ommen GJ,Dorsman JC

更新日期：2012-06-01 00:00:00

abstract：:Parkinson disease (PD) is a systemic disease with variegated non-motor deficits and neurological symptoms, including impaired olfaction, autonomic failure, cognitive impairment and psychiatric symptoms, in addition to the classical motor symptoms. Many non-motor symptoms appear before or in parallel with motor deficit...

journal_title：Neurobiology of disease

authors： Ferrer I,López-Gonzalez I,Carmona M,Dalfó E,Pujol A,Martínez A

更新日期：2012-06-01 00:00:00

abstract：:Signs or symptoms of impaired autonomic regulation of circulation often attend Parkinson disease (PD). This review covers biomarkers and mechanisms of autonomic cardiovascular abnormalities in PD and related alpha-synucleinopathies. The clearest clinical laboratory correlate of dysautonomia in PD is loss of myocardial...

journal_title：Neurobiology of disease

authors： Jain S,Goldstein DS

更新日期：2012-06-01 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：:Schizophrenia is a complex neuropsychiatric disorder that involves disturbances in neural circuitry and synaptic function. The exquisite network architecture and capacity for discreet post-synaptic remodeling of neurons requires coordination by an elaborate intracellular network of molecular signal transduction system...

journal_title：Neurobiology of disease

authors： Beveridge NJ,Cairns MJ

更新日期：2012-05-01 00:00:00

abstract：:Patients with Krabbe disease, a genetic demyelinating syndrome caused by deficiency of galactosyl-ceramidase and the resulting accumulation of galactosyl-sphingolipids, develop signs of a dying-back axonopathy compounded by a deficiency of large-caliber axons. Here, we show that axonal caliber in Twitcher mice, an ani...

journal_title：Neurobiology of disease

authors： Cantuti-Castelvetri L,Zhu H,Givogri MI,Chidavaenzi RL,Lopez-Rosas A,Bongarzone ER

更新日期：2012-05-01 00:00:00

abstract：:Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by an excessive expansion of a CAG trinucleotide repeat in the gene encoding the protein huntingtin, resulting in an elongated stretch of glutamines near the N-terminus of the protein. Here we report the derivation of a collection of ...

journal_title：Neurobiology of disease

authors： Castiglioni V,Onorati M,Rochon C,Cattaneo E

更新日期：2012-04-01 00:00:00

abstract：:Glutamate-induced delayed calcium dysregulation (DCD) is a causal factor leading to neuronal death. The mechanism of DCD is not clear but Ca2+ influx via N-methyl-d-aspartate receptors (NMDAR) and/or the reverse plasmalemmal Na+/Ca2+ exchanger (NCXrev) could be involved in DCD. However, the extent to which NMDAR and N...

journal_title：Neurobiology of disease

authors： Brittain MK,Brustovetsky T,Sheets PL,Brittain JM,Khanna R,Cummins TR,Brustovetsky N

更新日期：2012-04-01 00:00:00

abstract：:Friedreich ataxia (FRDA) is an autosomal recessive neurodegenerative disorder caused by a dynamic GAA repeat expansion mutation within intron 1 of the FXN gene. Studies of mouse models for other trinucleotide repeat (TNR) disorders have revealed an important role of mismatch repair (MMR) proteins in TNR instability. T...

journal_title：Neurobiology of disease

authors： Ezzatizadeh V,Pinto RM,Sandi C,Sandi M,Al-Mahdawi S,Te Riele H,Pook MA

更新日期：2012-04-01 00:00:00

abstract：:CLN5 disease, late infantile variant phenotype neuronal ceroid lipofuscinosis, is a severe neurodegenerative disease caused by mutations in the CLN5 gene, which encodes a lysosomal protein of unknown function. Cln5-deficiency in mice leads to loss of thalamocortical neurons, and glial activation, but the underlying me...

journal_title：Neurobiology of disease

authors： Schmiedt ML,Blom T,Blom T,Kopra O,Wong A,von Schantz-Fant C,Ikonen E,Kuronen M,Jauhiainen M,Cooper JD,Jalanko A

更新日期：2012-04-01 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：:Early exposure to general anesthesia (GA) causes developmental neuroapoptosis in the mammalian brain and long-term cognitive impairment. Recent evidence suggests that GA also causes functional and morphological impairment of the immature neuronal mitochondria. Injured mitochondria could be a significant source of reac...

journal_title：Neurobiology of disease

authors： Boscolo A,Starr JA,Sanchez V,Lunardi N,DiGruccio MR,Ori C,Erisir A,Trimmer P,Bennett J,Jevtovic-Todorovic V

更新日期：2012-03-01 00:00:00

abstract：:Niemann-Pick disease type C (NPC) is an inherited lysosomal storage disease characterised by accumulation of cholesterol and glycosphingolipids. NPC patients suffer a progressive neurodegenerative phenotype presenting with motor dysfunction, mental retardation and cognitive decline. To examine the onset and progressio...

journal_title：Neurobiology of disease

authors： Pressey SN,Smith DA,Wong AM,Platt FM,Cooper JD

更新日期：2012-03-01 00:00:00

abstract：:Pathological oxygen deprivation inhibits prolyl hydroxylase (PHD) activity and stimulates a protective cellular oxygen-sensing response in part through the stabilization and activation of the Hypoxia Inducible Factor (HIF) 1α transcription factor. The present investigation tested the therapeutic potential of enhanced ...

journal_title：Neurobiology of disease

authors： Ogle ME,Gu X,Espinera AR,Wei L

更新日期：2012-02-01 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：:Decreased release probability (Pr) and increased failure rate for monosynaptic inhibitory postsynaptic currents (IPSCs) indicate abnormalities in presynaptic inhibitory terminals on pyramidal (Pyr) neurons of the undercut (UC) model of posttraumatic epileptogenesis. These indices of inhibition are normalized in high [...

journal_title：Neurobiology of disease

authors： Faria LC,Parada I,Prince DA

更新日期：2012-02-01 00:00:00

abstract：:β-Amyloid (Aβ) plaques in Alzheimer (AD) brains are surrounded by severe dendritic and axonal changes, including local spine loss, axonal swellings and distorted neurite trajectories. Whether and how plaques induce these neuropil abnormalities remains unknown. We tested the hypothesis that oligomeric assemblies of Aβ,...

journal_title：Neurobiology of disease

authors： DaRocha-Souto B,Coma M,Pérez-Nievas BG,Scotton TC,Siao M,Sánchez-Ferrer P,Hashimoto T,Fan Z,Hudry E,Barroeta I,Serenó L,Rodríguez M,Sánchez MB,Hyman BT,Gómez-Isla T

更新日期：2012-01-01 00:00:00

abstract：:Heterozygous in frame duplications of the PHOX2B gene, leading to polyalanine (polyAla) expansions ranging from +5 to +13 residues of a 20-alanine stretch, have been identified in the vast majority of patients affected with Congenital Central Hypoventilation Syndrome (CCHS), a rare neurocristopathy characterized by ab...

journal_title：Neurobiology of disease

authors： Di Zanni E,Bachetti T,Parodi S,Bocca P,Prigione I,Di Lascio S,Fornasari D,Ravazzolo R,Ceccherini I

更新日期：2012-01-01 00:00:00

abstract：:The molecular mechanisms of major mental illnesses, such as schizophrenia and bipolar disorder, are unclear. To address this fundamental question, many groups have studied molecular expression profiles in postmortem brains and other tissues from patients compared with those from normal controls. Development of unbiase...

journal_title：Neurobiology of disease

authors： Lin CY,Sawa A,Jaaro-Peled H

更新日期：2012-01-01 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：:Painful diabetic neuropathy (PDN) is a common, yet devastating complication of type 2 diabetes. At this time, there is no objective test for diagnosing PDN. In the current study, we measured the peptidergic intraepidermal nerve fiber densities (IENFD) from hind paws of the db/db mouse, an animal model for type 2 diabe...

journal_title：Neurobiology of disease

authors： Cheng HT,Dauch JR,Hayes JM,Yanik BM,Feldman EL

更新日期：2012-01-01 00:00:00

abstract：:The use of energy substrates, such as lactate and pyruvate, has been shown to improve synaptic function when administered during glucose deprivation. In the present study, we investigated whether prolonged incubation with monocarboxylate (pyruvate or lactate) prior rather than during glucose deprivation can also susta...

journal_title：Neurobiology of disease

authors： Shetty PK,Sadgrove MP,Galeffi F,Turner DA

更新日期：2012-01-01 00:00:00

abstract：:Mitochondrial dysfunction plays an important role in the pathogenesis of neurodegenerative diseases, numerous other disease states and senescence. The ability to monitor reactive oxygen species (ROS) within tissues and over time in animal model systems is of significant research value. Recently, redox-sensitive fluore...

journal_title：Neurobiology of disease

authors： Liu Z,Celotto AM,Romero G,Wipf P,Palladino MJ

更新日期：2012-01-01 00:00:00

abstract：UNLABELLED:Mitochondrial dysfunction is involved in the pathogenesis of motor neuron degeneration in the G93A mutant transgenic (tgmSOD1) animal model of ALS. However, it is unknown whether mitochondriopathy is a primary or secondary event. We isolated brain (BM) and spinal cord (SCM) mitochondria from 2 month old pres...

journal_title：Neurobiology of disease

authors： Panov A,Kubalik N,Zinchenko N,Hemendinger R,Dikalov S,Bonkovsky HL

更新日期：2011-10-01 00:00:00

abstract：:Transient receptor potential vanilloid 1 (TRPV1) channels are involved in several inflammatory diseases. However, their action is still controversial, and both pro-inflammatory and anti-inflammatory roles have been described. We used a strain of TRPV1-KO mice to characterize the role of these channels in experimental ...

journal_title：Neurobiology of disease

authors： Musumeci G,Grasselli G,Rossi S,De Chiara V,Musella A,Motta C,Studer V,Bernardi G,Haji N,Sepman H,Fresegna D,Maccarrone M,Mandolesi G,Centonze D

更新日期：2011-09-01 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：:Insulin deficiency may contribute toward the neurological deficits of diabetic polyneuropathy (DPN). In particular, the unique trophic properties of insulin, acting on sensory neuron and axon receptors offer an approach toward reversing loss of skin axons that develops during diabetes. Here we examined how local cutan...

journal_title：Neurobiology of disease

authors： Guo G,Kan M,Martinez JA,Zochodne DW

更新日期：2011-08-01 00:00:00

abstract：:The inwardly-rectifying potassium channel Kir4.1 is a major player in the astrocyte-mediated regulation of [K(+)](o) in the brain, which is essential for normal neuronal activity and synaptic functioning. KCNJ10, encoding Kir4.1, has been recently linked to seizure susceptibility in humans and mice, and is a possible ...

journal_title：Neurobiology of disease

authors： Sicca F,Imbrici P,D'Adamo MC,Moro F,Bonatti F,Brovedani P,Grottesi A,Guerrini R,Masi G,Santorelli FM,Pessia M

更新日期：2011-07-01 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：:Huntington's disease (HD) is a progressive neurodegenerative genetic disorder which leads to motor, cognitive and psychiatric disturbances. The primary neuropathological hallmark is atrophy of the striatum. Cannabinoid CB1 receptors (CB1Rs) are particularly enriched in the striatum and previous works indicate their ea...

journal_title：Neurobiology of disease

authors： Mievis S,Blum D,Ledent C

更新日期：2011-06-01 00:00:00

abstract：:Nicotinamide adenine dinucleotide phosphate oxidase (NOX) is widely expressed in brain tissue including neurons, glia, and endothelia in neurovascular units. It is a major source of oxidants in the post-ischemic brain and significantly contributes to ischemic brain damage. Inflammation occurs after brain ischemia and ...

journal_title：Neurobiology of disease

authors： Chen H,Kim GS,Okami N,Narasimhan P,Chan PH

更新日期：2011-06-01 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：:Carriers of FMR1 premutation alleles have 55-200 CGG repeats in the 5' untranslated region of the gene. These individuals are at risk for fragile X associated primary ovarian insufficiency (females) and, in late life, fragile X associated tremor and ataxia syndrome (males, and to a lesser extent, females). Premutation...

journal_title：Neurobiology of disease

authors： Qin M,Entezam A,Usdin K,Huang T,Liu ZH,Hoffman GE,Smith CB

更新日期：2011-04-01 00:00:00

abstract：:The neuronal ceroid lipofuscinoses (NCLs, Batten disease) are characterized by progressive neurodegeneration resulting in widespread brain atrophy. Each form is assumed to be the consequence of some universal intracellular event; however, time course studies on the cerebral cortex of a sheep model of the CLN6 form rev...

journal_title：Neurobiology of disease

authors： Kay GW,Jay NP,Palmer DN

更新日期：2011-03-01 00:00:00

abstract：:The prevalence of central nervous system (CNS) neurologic dysfunction associated with human immunodeficiency virus (HIV) infection continues to increase, despite the use of antiretroviral therapy. Previous work has focused on the deleterious effects of HIV on mature neurons and on development of neuroprotective strate...

journal_title：Neurobiology of disease

authors： Lee MH,Wang T,Jang MH,Steiner J,Haughey N,Ming GL,Song H,Nath A,Venkatesan A

更新日期：2011-03-01 00:00:00

abstract：OBJECTIVE:In Parkinson's disease, chronic striatal dopamine depletion results in over-activity and under-activity of the indirect and direct striatal output pathways respectively. In this study, we investigated changes in the function of glutamatergic cortico-striatal synapses that contribute to abnormalities in striat...

journal_title：Neurobiology of disease

authors： Warre R,Thiele S,Talwar S,Kamal M,Johnston TH,Wang S,Lam D,Lo C,Khademullah CS,Perera G,Reyes G,Sun XS,Brotchie JM,Nash JE

更新日期：2011-03-01 00:00:00

abstract：:Mesenchymal stem cells (MSCs) are reported to possess immunomodulatory properties. Previous reports have demonstrated the beneficial effects of MSC-transplantation in focal cerebral ischemia animal models. In this study, we have investigated the neuroimmunomodulatory functions of human MSCs, transplanted in a rat foca...

journal_title：Neurobiology of disease

authors： Sheikh AM,Nagai A,Wakabayashi K,Narantuya D,Kobayashi S,Yamaguchi S,Kim SU

更新日期：2011-03-01 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