abstract：:Midlife cognitive decline is now recognized as a factor of poor prognosis for late-life dementia. Although an epidemiological link has been suggested with high fat diet (HFD)-induced metabolic disorders, the effect of a long period of HFD on midlife cerebrovascular and cognitive functions remains unproven. A cohort of...

journal_title：Neurobiology of disease

authors： Pétrault O,Pétrault M,Ouk T,Bordet R,Bérézowski V,Bastide M

更新日期：2019-10-01 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：: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 interest in the pedunculopontine tegmental nucleus (PPTg), a structure located in the brainstem at the level of the pontomesencephalic junction, has greatly increased in recent years because it is involved in the regulation of physiological functions that fail in Parkinson's disease and because it is a promising t...

journal_title：Neurobiology of disease

authors： Vitale F,Capozzo A,Mazzone P,Scarnati E

更新日期：2019-08-01 00:00:00

abstract：:The clinical trials employing neuroprotectants targeting single, early pathogenic mechanisms in stroke have so far been barely successful. We found in human postmortem stroke brains that in addition to apoptosis, necroptosis also contributed to neuronal damage. Thus, a new strategy targeting both mechanisms might be n...

journal_title：Neurobiology of disease

authors： Han F,Guan X,Guo W,Lu B

更新日期：2019-07-01 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：:Apoptosis is triggered in the developing mammalian brain by sedative, anesthetic or antiepileptic drugs during late gestation and early life. Whether human children are vulnerable to this toxicity mechanism remains unknown, as there are no imaging techniques to capture it. Apoptosis is characterized by distinct struct...

journal_title：Neurobiology of disease

authors： Rosado-Mendez IM,Noguchi KK,Castañeda-Martinez L,Kirvassilis G,Wang SH,Manzella F,Swiney BS,Masuoka K,Capuano S III2,Brunner KG,Crosno K,Guerrero QW,Whitson H,Brambrink A,Simmons HS,Mejia AF,Zagzebski JA,Hall TJ,Ikono

更新日期：2019-07-01 00:00:00

abstract：:Beta power suppression in the basal ganglia is stronger during movements that require high force levels and high movement effort but it has been difficult to dissociate the two. We recorded scalp EEG and basal ganglia local field potentials in Parkinson's disease patients (11 STN, 7 GPi) ON and OFF dopaminergic medica...

journal_title：Neurobiology of disease

authors： Fischer P,Pogosyan A,Green AL,Aziz TZ,Hyam J,Foltynie T,Limousin P,Zrinzo L,Samuel M,Ashkan K,Da Lio M,De Cecco M,Fornaser A,Brown P,Tan H

更新日期：2019-07-01 00:00:00

abstract：:Recent large-scale genetic studies have allowed for the first glimpse of the effects of common genetic variability in dementia with Lewy bodies (DLB), identifying risk variants with appreciable effect sizes. However, it is currently well established that a substantial portion of the genetic heritable component of comp...

journal_title：Neurobiology of disease

authors： Guerreiro R,Escott-Price V,Hernandez DG,Kun-Rodrigues C,Ross OA,Orme T,Neto JL,Carmona S,Dehghani N,Eicher JD,Shepherd C,Parkkinen L,Darwent L,Heckman MG,Scholz SW,Troncoso JC,Pletnikova O,Dawson T,Rosenthal L,Ansor

更新日期：2019-07-01 00:00:00

abstract：:Ample evidence has surfaced documenting the neurotoxic effects of various general anesthetic (GA) agents in the mammalian brain when administered at critical periods of synaptogenesis. However, little is known about how this neurotoxic insult affects persisting neuronal excitability after the initial exposure. Here we...

journal_title：Neurobiology of disease

authors： Woodward TJ,Timic Stamenic T,Todorovic SM

更新日期：2019-07-01 00:00:00

abstract：AIM:Recent studies revealed that pharmacological modulation of NAE-hydrolyzing acid amidase (NAAA) can be achieved with PEA oxazoline (PEA-OXA). Hence, the aim of the present work was to thoroughly evaluate the anti-inflammatory and neuroprotective effects of PEA-OXA in an experimental model of vascular dementia (VaD) ...

journal_title：Neurobiology of disease

authors： Impellizzeri D,Siracusa R,Cordaro M,Crupi R,Peritore AF,Gugliandolo E,D'Amico R,Petrosino S,Evangelista M,Di Paola R,Cuzzocrea S

更新日期：2019-05-01 00:00:00

abstract：:The death of mature oligodendrocytes (OLs) leads to demyelination in the central nervous system (CNS) and subsequently to functional deficits. Remyelination requires the differentiation of oligodendrocyte progenitor cells (OPCs) into myelinating OLs, which in the CNS with neurodegenerative diseases such as multiple sc...

journal_title：Neurobiology of disease

authors： Zhang J,Zhang ZG,Lu M,Zhang Y,Shang X,Chopp M

更新日期：2019-05-01 00:00:00

abstract：:TDP-43 proteinopathy is very prevalent among the elderly (affecting at least 25% of individuals over 85 years of age) and is associated with substantial cognitive impairment. Risk factors implicated in age-related TDP-43 proteinopathy include commonly inherited gene variants, comorbid Alzheimer's disease pathology, an...

journal_title：Neurobiology of disease

authors： Nelson PT,Gal Z,Wang WX,Niedowicz DM,Artiushin SC,Wycoff S,Wei A,Jicha GA,Fardo DW

更新日期：2019-05-01 00:00:00

abstract：BACKGROUND:Epidemiological studies suggest that the risk of neurodevelopmental disorders such as autism spectrum disorder (ASD) and schizophrenia is increased by prenatal exposure to viral or bacterial infection during pregnancy. It is still unclear how activation of the maternal immune response interacts with underlyi...

journal_title：Neurobiology of disease

authors： Amodeo DA,Lai CY,Hassan O,Mukamel EA,Behrens MM,Powell SB

更新日期：2019-05-01 00:00:00

abstract：:There is evidence that cognitive decline in Alzheimer's disease (AD) results from deficiencies in synaptic communication (e.g., loss of mushroom-shaped 'memory spines') and neurodegenerative processes. This might be treated with sigma-1 receptor (S1R) agonists, which are broadly neuroprotective and modulate synaptic p...

journal_title：Neurobiology of disease

authors： Ryskamp D,Wu L,Wu J,Kim D,Rammes G,Geva M,Hayden M,Bezprozvanny I

更新日期：2019-04-01 00:00:00

abstract：:Deep brain stimulation of certain target structures within the basal ganglia is an effective therapy for the management of the motor symptoms of Parkinson's disease. However, its mechanisms, as well as the pathophysiology of Parkinson's disease, are varied and complex. The classical model of Parkinson's disease states...

journal_title：Neurobiology of disease

authors： Milosevic L,Gramer R,Kim TH,Algarni M,Fasano A,Kalia SK,Hodaie M,Lozano AM,Popovic MR,Hutchison WD

更新日期：2019-04-01 00:00:00

abstract：:Aggregation of α-synuclein (α-syn) is neuropathologically and genetically linked to Parkinson's disease (PD). Since stereotypic cell-to-cell spreading of α-syn pathology is believed to contribute to disease progression, immunotherapy with antibodies directed against α-syn is considered a promising therapeutic approach...

journal_title：Neurobiology of disease

authors： Weihofen A,Liu Y,Arndt JW,Huy C,Quan C,Smith BA,Baeriswyl JL,Cavegn N,Senn L,Su L,Marsh G,Auluck PK,Montrasio F,Nitsch RM,Hirst WD,Cedarbaum JM,Pepinsky RB,Grimm J,Weinreb PH

更新日期：2019-04-01 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：:No single-omic approach completely elucidates the multitude of alterations taking place in Alzheimer's disease (AD). Here, we coupled transcriptomic and phosphoproteomic approaches to determine the temporal sequence of changes in mRNA, protein, and phosphopeptide expression levels from human temporal cortical samples,...

journal_title：Neurobiology of disease

authors： Marttinen M,Paananen J,Neme A,Mitra V,Takalo M,Natunen T,Paldanius KMA,Mäkinen P,Bremang M,Kurki MI,Rauramaa T,Leinonen V,Soininen H,Haapasalo A,Pike I,Hiltunen M

更新日期：2019-04-01 00:00:00

abstract：:Neuropathological findings in the amygdala obtained from patients with mesial temporal lobe epilepsy (MTLE) indicate varying degrees of histopathological alterations, such as neuronal loss and gliosis. The mechanisms underlying cellular damage in the amygdala of patients with MTLE have not been fully elucidated. In th...

journal_title：Neurobiology of disease

authors： Jafarian M,Modarres Mousavi SM,Alipour F,Aligholi H,Noorbakhsh F,Ghadipasha M,Gharehdaghi J,Kellinghaus C,Kovac S,Khaleghi Ghadiri M,Meuth SG,Speckmann EJ,Stummer W,Gorji A

更新日期：2019-04-01 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：:Mutations in the gene encoding the RNA-binding protein TDP-43 cause amyotrophic lateral sclerosis (ALS), clinically and pathologically indistinguishable from the majority of 'sporadic' cases of ALS, establishing altered TDP-43 function and distribution as a primary mechanism of neurodegeneration. Transgenic mouse mode...

journal_title：Neurobiology of disease

authors： Gordon D,Dafinca R,Scaber J,Alegre-Abarrategui J,Farrimond L,Scott C,Biggs D,Kent L,Oliver PL,Davies B,Ansorge O,Wade-Martins R,Talbot K

更新日期：2019-01-01 00:00:00

abstract：:Huntington's disease (HD) is a hereditary neurodegenerative disorder caused by an expansion of a CAG repeat in the huntingtin (htt) gene, which results in an aberrant form of the protein (mhtt). This leads to motor and cognitive deficits associated with corticostriatal and hippocampal alterations. The levels of STriat...

journal_title：Neurobiology of disease

authors： García-Forn M,Martínez-Torres S,García-Díaz Barriga G,Alberch J,Milà M,Azkona G,Pérez-Navarro E

更新日期：2018-12-01 00:00:00

abstract：:GPNMB is a glycoprotein observed upon tissue damage and inflammation and is associated with astrocytes, microglia, and macrophages. Gene variations in GPNMB are linked with Parkinson's disease (PD) risk, and changes in protein levels of GPNMB have been found in lysosomal storage disorders, including Gaucher's disease ...

journal_title：Neurobiology of disease

authors： Moloney EB,Moskites A,Ferrari EJ,Isacson O,Hallett PJ

更新日期：2018-12-01 00:00:00

abstract：:Freezing of gait (FOG) is a devastating axial motor symptom in Parkinson's disease (PD) leading to falls, institutionalization, and even death. The response of FOG to dopaminergic medication and deep brain stimulation (DBS) is complex, variable, and yet to be optimized. Fundamental gaps in the knowledge of the underly...

journal_title：Neurobiology of disease

authors： Anidi C,O'Day JJ,Anderson RW,Afzal MF,Syrkin-Nikolau J,Velisar A,Bronte-Stewart HM

更新日期：2018-12-01 00:00:00

abstract：:Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is a rare type of leukodystrophy caused by mutations in either MLC1 or GLIALCAM genes. Previous work indicated that chloride currents mediated by the volume-regulated anion channel (VRAC) and ClC-2 channels were affected in astrocytes deficient in either...

journal_title：Neurobiology of disease

authors： Elorza-Vidal X,Sirisi S,Gaitán-Peñas H,Pérez-Rius C,Alonso-Gardón M,Armand-Ugón M,Lanciotti A,Brignone MS,Prat E,Nunes V,Ambrosini E,Gasull X,Estévez R

更新日期：2018-11-01 00:00:00

abstract：:Alpha-synuclein (aSyn) is the major protein component of Lewy bodies and Lewy neurites, the typical pathological hallmarks in Parkinson's disease (PD) and Dementia with Lewy bodies. aSyn is capable of inducing transcriptional deregulation, but the precise effect of specific aSyn mutants associated with familial forms ...

journal_title：Neurobiology of disease

authors： Paiva I,Jain G,Lázaro DF,Jerčić KG,Hentrich T,Kerimoglu C,Pinho R,Szegő ÈM,Burkhardt S,Capece V,Halder R,Islam R,Xylaki M,Caldi Gomes LA,Roser AE,Lingor P,Schulze-Hentrich JM,Borovečki F,Fischer A,Outeiro TF

更新日期：2018-11-01 00:00:00

abstract：:Thermal hyperpnea, a pattern of breathing during hyperthermia that is characterized by an increase in tidal volume as well as breathing frequency, is known to lead to respiratory alkalosis. Thermal hyperpnea-induced respiratory alkalosis is linked to febrile seizures (FS). The heat-sensitive transient receptor potenti...

journal_title：Neurobiology of disease

authors： Barrett KT,Roy A,Rivard KB,Wilson RJA,Scantlebury MH

更新日期：2018-11-01 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：:Amyloid-β is a peptide released by synapses in physiological conditions and its pathological accumulation in brain structures necessary for memory processing represents a key toxic hallmark underlying Alzheimer's disease. The oligomeric form of Amyloid-β (Aβο) is now believed to represent the main Amyloid-β species af...

journal_title：Neurobiology of disease

authors： Kootar S,Frandemiche ML,Dhib G,Mouska X,Lorivel T,Poupon-Silvestre G,Hunt H,Tronche F,Bethus I,Barik J,Marie H

更新日期：2018-10-01 00:00:00

abstract：:The motor cortex and subthalamic nucleus (STN) of patients with Parkinson's disease (PD) exhibit abnormally high levels of electrophysiological oscillations in the ~12-35 Hz beta-frequency range. Recent studies have shown that beta is partly carried forward to regulate future motor states in the healthy condition, sug...

journal_title：Neurobiology of disease

authors： Polar CA,Gupta R,Lehmkuhle MJ,Dorval AD

更新日期：2018-09-01 00:00:00

abstract：:Multiple sclerosis (MS) is the prototypical inflammatory disease of the central nervous system (CNS), leading to multifocal demyelination and neurodegeneration. The etiology of this incurable disease is unknown and remains a matter of intensive research. The possibility that microbial infections, such as viruses or ba...

journal_title：Neurobiology of disease

authors： Alonso R,Fernández-Fernández AM,Pisa D,Carrasco L

更新日期：2018-09-01 00:00:00

abstract：:Exaggerated activity in the beta band (13-35 Hz) is a hallmark of basal ganglia signals in patients with Parkinson's disease (PD). Beta activity however is not constantly elevated, but comes in bursts. In previous work we showed that the longer beta bursts are maintained, the more the oscillatory synchronisation withi...

journal_title：Neurobiology of disease

authors： Tinkhauser G,Torrecillos F,Duclos Y,Tan H,Pogosyan A,Fischer P,Carron R,Welter ML,Karachi C,Vandenberghe W,Nuttin B,Witjas T,Régis J,Azulay JP,Eusebio A,Brown P

更新日期：2018-09-01 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：:Here we report a gain in function for mutant (mt) superoxide dismutase I (SOD1), a cause of familial amyotrophic lateral sclerosis (FALS), wherein small soluble oligomers of mtSOD1 acquire a membrane toxicity. Phosphatidylglycerol (PG) lipid domains are selectively targeted, which could result in membrane damage or "t...

journal_title：Neurobiology of disease

authors： Riehm JJ,Wang L,Ghadge G,Teng M,Correa AM,Marks JD,Roos RP,Allen MJ

更新日期：2018-07-01 00:00:00

abstract：:Biallelic nonsense mutations of SYNE1 underlie a variable array of cerebellar and non-cerebellar pathologies of unknown molecular etiology. SYNE1 encodes multiple isoforms of Nesprin1 that associate with the nuclear envelope, with large cerebellar synapses and with ciliary rootlets of photoreceptors. Using two novel m...

journal_title：Neurobiology of disease

authors： Potter C,Razafsky D,Wozniak D,Casey M,Penrose S,Ge X,Mahjoub MR,Hodzic D

更新日期：2018-07-01 00:00:00

abstract：:Inhibition of mitochondrial axonal trafficking by amyloid beta (Aβ) peptides has been implicated in early pathophysiology of Alzheimer's Disease (AD). Yet, it remains unclear whether the loss of motility inevitably induces the loss of mitochondrial function, and whether restoration of axonal trafficking represents a v...

journal_title：Neurobiology of disease

authors： Zhang L,Trushin S,Christensen TA,Tripathi U,Hong C,Geroux RE,Howell KG,Poduslo JF,Trushina E

更新日期：2018-06-01 00:00:00

abstract：:Vascular cognitive impairment is a major cause of dementia caused by chronic hypoxia, producing progressive damage to white matter (WM) secondary to blood-brain barrier (BBB) opening and vascular dysfunction. Tight junction proteins (TJPs), which maintain BBB integrity, are lost in acute ischemia. Although angiogenesi...

journal_title：Neurobiology of disease

authors： Yang Y,Kimura-Ohba S,Thompson JF,Salayandia VM,Cossé M,Raz L,Jalal FY,Rosenberg GA

更新日期：2018-06-01 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：:Neuropeptide Y (NPY) is an important 36 amino acid peptide that is abundantly expressed in the mammalian CNS and is known to be an endogenous modulator of seizure activity, including in rat models of Genetic Generalised Epilepsy (GGE) with absence seizures. Studies have shown that viral-mediated "gene therapy" with ov...

journal_title：Neurobiology of disease

authors： Powell KL,Fitzgerald X,Shallue C,Jovanovska V,Klugmann M,Von Jonquieres G,O'Brien TJ,Morris MJ

更新日期：2018-05-01 00:00:00