Cholesterol retention in Alzheimer's brain is responsible for high beta- and gamma-secretase activities and Abeta production.

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：:Human immunodeficiency virus (HIV) infection continues to rise in drug-abusing populations and causes a dementing illness in a subset of individuals. Factors contributing to the development of dementia in this population remain unknown. We found that HIV-infected individuals with the E4 allele of Apolipoprotein E (Apo...

journal_title：Neurobiology of disease

authors： Turchan-Cholewo J,Liu Y,Gartner S,Reid R,Jie C,Peng X,Chen KC,Chauhan A,Haughey N,Cutler R,Mattson MP,Pardo C,Conant K,Sacktor N,McArthur JC,Hauser KF,Gairola C,Nath A

更新日期：2006-07-01 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：:Alzheimer's disease (AD) involves changes in both lipid and RNA metabolism, but it remained unknown if these differences associate with AD's cognition and/or post-mortem neuropathology indices. Here, we report RNA-sequencing evidence of inter-related associations between lipid processing, cognition level, and AD neuro...

journal_title：Neurobiology of disease

authors： Barbash S,Garfinkel BP,Maoz R,Simchovitz A,Nadorp B,Guffanti A,Bennett ER,Nadeau C,Türk A,Paul L,Reda T,Li Y,Buchman AS,Greenberg DS,Seitz A,Bennett DA,Giavalisco P,Soreq H

更新日期：2017-10-01 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：:Evidence of the gut microbiota influencing neurodegenerative diseases has been reported for several neural diseases. However, there is little insight regarding the relationship between the gut microbiota and prion disease. Here, using fecal samples of 12 prion-infected mice and 25 healthy controls, we analyzed the str...

journal_title：Neurobiology of disease

authors： Yang D,Zhao D,Shah SZA,Wu W,Lai M,Zhang X,Li J,Guan Z,Zhao H,Li W,Gao H,Zhou X,Yang L

更新日期：2020-02-01 00:00:00

abstract：:Huntington's disease (HD) is a genetic neurodegenerative disease characterized by movement disorders, cognitive decline and neuropsychiatric symptoms. HD is caused by expanded CAG tract within the coding region of Huntingtin protein. Despite major insights into the molecular mechanisms leading to HD, no effective cure...

journal_title：Neurobiology of disease

authors： Dupont P,Besson MT,Devaux J,Liévens JC

更新日期：2012-08-01 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：:The TATA-box binding protein associated factor 1 (TAF1) is part of the TFIID complex that plays a key role during the initiation of transcription. Variants of TAF1 are associated with neurodevelopmental disorders. Previously, we found that CRISPR/Cas9 based editing of the TAF1 gene disrupts the morphology of the cereb...

journal_title：Neurobiology of disease

authors： Dhanalakshmi C,Janakiraman U,Moutal A,Fukunaga K,Khanna R,Nelson MA

更新日期：2021-02-01 00:00:00

abstract：:Optic neuritis is a frequent and early symptom of multiple sclerosis (MS). Conventional magnetic resonance (MR) techniques provide means to assess multiple MS-related pathologies, including axonal injury, demyelination, and inflammation. A method to directly and non-invasively probe white-matter function could further...

journal_title：Neurobiology of disease

authors： Lin TH,Spees WM,Chiang CW,Trinkaus K,Cross AH,Song SK

更新日期：2014-07-01 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：:Fragile X Syndrome (FXS) is a heritable form of mental retardation caused by a non-coding trinucleotide expansion of the FMR1 gene leading to loss of expression of this RNA binding protein. Mutations in this gene are strongly linked to enhanced Group I metabotropic glutamate receptor (mGluR) signaling. A recent report...

journal_title：Neurobiology of disease

authors： Straiker A,Min KT,Mackie K

更新日期：2013-08-01 00:00:00

abstract：:The present study aimed to evaluate the therapeutic potential of clioquinol (CQ), a metal chelator, on multiple sclerosis pathogenesis. Experimental autoimmune encephalomyelitis was induced by immunization with myelin oligodendrocyte glycoprotein (MOG(35-55)) in female mice. Three weeks after the initial immunization,...

journal_title：Neurobiology of disease

authors： Choi BY,Jang BG,Kim JH,Seo JN,Wu G,Sohn M,Chung TN,Suh SW

更新日期：2013-06-01 00:00:00

abstract：:The optic nerve crush injury is a well-accepted model to study the mechanisms of axonal regeneration after trauma in the CNS. The infection of retinal ganglion cells (RGCs) with an adeno-associated virus serotype 2 - ciliary neurotrophic factor (AAV2.CNTF) was previously shown to stimulate axonal regeneration. However...

journal_title：Neurobiology of disease

authors： Pernet V,Joly S,Dalkara D,Jordi N,Schwarz O,Christ F,Schaffer DV,Flannery JG,Schwab ME

更新日期：2013-03-01 00:00:00

abstract：:Disturbances in central serotonergic systems have been hypothesized to be involved in seasonal affective disorder (SAD). Association between SAD and the shorter allele of the serotonin transporter promoter repeat length polymorphism (5-HTTLPR) has been reported in an American sample. We have genotyped 82 SAD patients ...

journal_title：Neurobiology of disease

authors： Johansson C,Smedh C,Partonen T,Pekkarinen P,Paunio T,Ekholm J,Peltonen L,Lichtermann D,Palmgren J,Adolfsson R,Schalling M

更新日期：2001-04-01 00:00:00

abstract：:Mutations in the voltage-gated sodium channel SCN1A are responsible for a number of seizure disorders including Generalized Epilepsy with Febrile Seizures Plus (GEFS+) and Severe Myoclonic Epilepsy of Infancy (SMEI). To determine the effects of SCN1A mutations on channel function in vivo, we generated a bacterial arti...

journal_title：Neurobiology of disease

authors： Tang B,Dutt K,Papale L,Rusconi R,Shankar A,Hunter J,Tufik S,Yu FH,Catterall WA,Mantegazza M,Goldin AL,Escayg A

更新日期：2009-07-01 00:00:00

abstract：:Charcot-Marie-Tooth disease (CMT) is a genetic disorder that can be caused by aberrations in >80 genes. CMT has heterogeneous modes of inheritance, including autosomal dominant, autosomal recessive, X-linked dominant, and X-linked recessive. Over 95% of cases are dominantly inherited. In this study, we investigated wh...

journal_title：Neurobiology of disease

authors： Lee JS,Chang EH,Koo OJ,Jwa DH,Mo WM,Kwak G,Moon HW,Park HT,Hong YB,Choi BO

更新日期：2017-04-01 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

abstract：:The epsilon4 allele of the apolipoprotein E gene (APOE) is associated with sporadic and familial late-onset Alzheimer's disease (AD). Oxidative stress is believed to play an important role in neuronal dysfunction and cell death in AD. We now provide evidence that in the hippocampus of AD, the level of thiobarbituric a...

journal_title：Neurobiology of disease

authors： Ramassamy C,Averill D,Beffert U,Theroux L,Lussier-Cacan S,Cohn JS,Christen Y,Schoofs A,Davignon J,Poirier J

更新日期：2000-02-01 00:00:00

abstract：BACKGROUND:Alzheimer's disease (AD) is the most common neurodegenerative disorder. Depositions of amyloid β peptide (Aβ) and tau protein are among the major pathological hallmarks of AD. Aβ and tau burden follows predictable spatial patterns during the progression of AD. Nevertheless, it remains obscure why certain bra...

journal_title：Neurobiology of disease

authors： Mendonça CF,Kuras M,Nogueira FCS,Plá I,Hortobágyi T,Csiba L,Palkovits M,Renner É,Döme P,Marko-Varga G,Domont GB,Rezeli M

更新日期：2019-10-01 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：:Ischemic stroke remains a leading cause of disability worldwide. Surviving patients often suffer permanent neurological impairments, and spontaneous recovery rarely occurs. However, observations that early-life brain injuries, including strokes, elicit less severe long-term functional impairments, compared to adults, ...

journal_title：Neurobiology of disease

authors： Teo L,Boghdadi AG,de Souza M,Bourne JA

更新日期：2018-03-01 00:00:00

abstract：:In addition to the substantial biological diversity among humans, our limited ability to reliably measure expression changes of small magnitude significantly reduces our capacity to obtain convergent sets of transcriptome data in postmortem brain. In particular, differences in the structure and sensitivity/reproducibi...

journal_title：Neurobiology of disease

authors： Hollingshead D,Lewis DA,Mirnics K

更新日期：2005-04-01 00:00:00

abstract：:Motor neuron degeneration in amyotrophic lateral sclerosis (ALS) is a form of apoptosis, but the mechanisms for this neuronal cell death are not known. We evaluated whether motor neuron degeneration in ALS is associated with changes in the levels and function of the apoptosis regulating protein p53. The protein levels...

journal_title：Neurobiology of disease

authors： Martin LJ

更新日期：2000-12-01 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：:Environmental toxicants and, in particular, pesticides have been implicated as risk factors in Parkinson's disease (PD). The purpose of this study was to determine if selective nigrostriatal degeneration could be reproduced by systemic exposure of mice to the widely used herbicide paraquat. Repeated intraperitoneal pa...

journal_title：Neurobiology of disease

authors： McCormack AL,Thiruchelvam M,Manning-Bog AB,Thiffault C,Langston JW,Cory-Slechta DA,Di Monte DA

更新日期：2002-07-01 00:00:00

abstract：:Amyotrophic lateral sclerosis (ALS) is a rapidly progressive and ultimately fatal neurodegenerative disease, caused by the loss of motor neurons in the brain and spinal cord. Although 10% of ALS cases are familial (FALS), the majority are sporadic (SALS) and probably associated to a multifactorial etiology. Currently ...

journal_title：Neurobiology of disease

authors： Aronica E,Baas F,Iyer A,ten Asbroek AL,Morello G,Cavallaro S

更新日期：2015-02-01 00:00:00

abstract：:The telomeric copy of the survival motor neuron gene (SMN1) is deleted or mutated in all spinal muscular atrophy (SMA) patients and these patients present mainly a loss in spinal motoneurons. Although studies performed in HeLa cells suggest that SMN may be involved in the biogenesis and possibly in recycling of splice...

journal_title：Neurobiology of disease

authors： Cisterni C,Kallenbach S,Jordier F,Bagnis C,Pettmann B

更新日期：2001-04-01 00:00:00

abstract：:Voltage-gated sodium channels (VGSCs) are essential for the generation and propagation of action potentials in electrically excitable cells. Dominant mutations in SCN1A, which encodes the Nav1.1 VGSC α-subunit, underlie several forms of epilepsy, including Dravet syndrome (DS) and genetic epilepsy with febrile seizure...

journal_title：Neurobiology of disease

authors： Dutton SB,Makinson CD,Papale LA,Shankar A,Balakrishnan B,Nakazawa K,Escayg A

更新日期：2013-01-01 00:00:00

abstract：:Alpha-synuclein containing cellular inclusions are a hallmark of Parkinson Disease, Lewy Body Dementia, and Multiple System Atrophy. A genome wide expression screen was performed in C. elegans overexpressing both wild-type and A53T human alpha-synuclein. 433 genes were up- and 67 genes down-regulated by statistical an...

journal_title：Neurobiology of disease

authors： Vartiainen S,Pehkonen P,Lakso M,Nass R,Wong G

更新日期：2006-06-01 00:00:00