Excitatory-inhibitory imbalance in Alzheimer's disease and therapeutic significance.

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：:Dravet syndrome is a severe infantile-onset epileptic encephalopathy which begins with febrile seizures and is caused by heterozygous loss-of-function mutations of the voltage-gated sodium channel gene SCN1A. We designed a CRISPR-based gene therapy for Scn1a-haplodeficient mice using multiple guide RNAs (gRNAs) in the...

journal_title：Neurobiology of disease

authors： Yamagata T,Raveau M,Kobayashi K,Miyamoto H,Tatsukawa T,Ogiwara I,Itohara S,Hensch TK,Yamakawa K

更新日期：2020-07-01 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：: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：:Human apolipoprotein E (apoE) exists in three isoforms: apoE2, apoE3 and apoE4. APOE ε4 is a major genetic risk factor for cardiovascular disease (CVD) and Alzheimer's disease (AD). ApoE mediates cholesterol metabolism by binding various receptors. The low-density lipoprotein receptor (LDLR) has a high affinity for ap...

journal_title：Neurobiology of disease

authors： Johnson LA,Olsen RH,Merkens LS,DeBarber A,Steiner RD,Sullivan PM,Maeda N,Raber J

更新日期：2014-04-01 00:00:00

abstract：:A wide spectrum of focal, regional, or diffuse structural brain abnormalities, collectively known as malformations of cortical development (MCDs), frequently manifest with intellectual disability (ID), epilepsy, and/or autistic spectrum disorder (ASD). As the acronym suggests, MCDs are perturbations of the normal arch...

journal_title：Neurobiology of disease

authors： Stouffer MA,Golden JA,Francis F

更新日期：2016-08-01 00:00:00

abstract：:The hyh mouse carrying a point mutation in the gene encoding for soluble N-ethylmaleimide-sensitive factor (NSF) attachment protein alpha (alpha-SNAP) develops inherited hydrocephalus. The investigation was designed to study: (i) the clinical evolution of hyh mice; (ii) factors other than the alpha-SNAP mutation that ...

journal_title：Neurobiology of disease

authors： Bátiz LF,Páez P,Jiménez AJ,Rodríguez S,Wagner C,Pérez-Fígares JM,Rodríguez EM

更新日期：2006-07-01 00:00:00

abstract：:The choroid plexus contains a major reservoir of macrophages poised for efficient delivery of virus and neurotoxins to the brain after infection by lentiviruses such as human or feline immunodeficiency virus (FIV). However, their contribution to neurotoxicity is poorly understood. Medium from FIV-infected, choroid ple...

journal_title：Neurobiology of disease

authors： Bragg DC,Boles JC,Meeker RB

更新日期：2002-03-01 00:00:00

abstract：:Neuroprotective functions of erythropoietin (Epo) are thought to involve a heteroreceptor composed of both Epo receptor (Epo-R) and common beta chain (betac). Here, we measured the response of hippocampal Epo system components (Epo, Epo-R and betac) during neurodegenerative processes following pilocarpine-induced stat...

journal_title：Neurobiology of disease

authors： Nadam J,Navarro F,Sanchez P,Moulin C,Georges B,Laglaine A,Pequignot JM,Morales A,Ryvlin P,Bezin L

更新日期：2007-02-01 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：:Previous studies have indicated that administration of glial cell line-derived neurotrophic factor (GDNF) counteracts neuronal death after stroke. However, in these studies damage was evaluated at most a few days after the insult. Here, we have explored the long-term consequences of two routes of GDNF delivery to the ...

journal_title：Neurobiology of disease

authors： Arvidsson A,Kirik D,Lundberg C,Mandel RJ,Andsberg G,Kokaia Z,Lindvall O

更新日期：2003-12-01 00:00:00

abstract：:Studies have showed that prenatal cocaine exposure (PCOC) can impair cognitive function and social behavior of the offspring; however, the mechanism underlying such effect is poorly understood. Insulin-like growth factor II (Igf-II), an imprinted gene, has a critical role in memory consolidation and enhancement. We hy...

journal_title：Neurobiology of disease

authors： Zhao Q,Hou J,Chen B,Shao X,Zhu R,Bu Q,Gu H,Li Y,Zhang B,Du C,Fu D,Kong J,Luo L,Long H,Li H,Deng Y,Zhao Y,Cen X

更新日期：2015-10-01 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：: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：:The focus on amyloid plaques and neurofibrillary tangles has yielded no Alzheimer's disease (AD) modifying treatments in the past several decades, despite successful studies in preclinical mouse models. This inconsistency has caused a renewed focus on improving the fidelity and reliability of AD mouse models, with dis...

journal_title：Neurobiology of disease

authors： Lewandowski CT,Maldonado Weng J,LaDu MJ

更新日期：2020-06-01 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：:TAR-DNA binding protein 43 (TDP-43) is a multifunctional RNA binding protein directly implicated in the etiology of amyotrophic lateral sclerosis (ALS). Previous studies have demonstrated that loss of TDP-43 function leads to intracellular accumulation of non-coding repetitive element transcripts and double-stranded R...

journal_title：Neurobiology of disease

authors： LaRocca TJ,Mariani A,Watkins LR,Link CD

更新日期：2019-12-01 00:00:00

abstract：:Fragile X syndrome (FXS) patients do not make the fragile X mental retardation protein (FMRP). The absence of FMRP causes dysregulated translation, abnormal synaptic plasticity and the most common form of inherited intellectual disability. But FMRP loss has minimal effects on memory itself, making it difficult to unde...

journal_title：Neurobiology of disease

authors： Radwan B,Dvorak D,Fenton AA

更新日期：2016-04-01 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：:Amyloid-beta (Abeta) accumulation in senile plaques is a hallmark of Alzheimer's disease (AD). Immunotherapy is a leading approach for amyloid clearance, despite the early termination of the Elan clinical trial with active immunization due to a few cases of meningoencephalitis. The mechanisms of immunotherapy-mediated...

journal_title：Neurobiology of disease

authors： Garcia-Alloza M,Ferrara BJ,Dodwell SA,Hickey GA,Hyman BT,Bacskai BJ

更新日期：2007-12-01 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：: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：:Down syndrome (DS) is the most common genetic disorder associated with mental retardation. It has been repeatedly shown that Ts65Dn mice, the major animal model for DS, have severe cognitive and synaptic plasticity dysfunctions caused by excessive inhibition in their temporal lobe structures. Here we employed a multid...

journal_title：Neurobiology of disease

authors： Begenisic T,Baroncelli L,Sansevero G,Milanese M,Bonifacino T,Bonanno G,Cioni G,Maffei L,Sale A

更新日期：2014-03-01 00:00:00

abstract：:Huntington disease (HD), a neurodegenerative disorder caused by an expanded CAG repeat in the HTT gene, remains without a treatment to modify the course of the illness. Lithium, a drug widely used for the treatment of bipolar disorder, has been shown to exert neuroprotective effects in a number of models of neurologic...

journal_title：Neurobiology of disease

authors： Pouladi MA,Brillaud E,Xie Y,Conforti P,Graham RK,Ehrnhoefer DE,Franciosi S,Zhang W,Poucheret P,Compte E,Maurel JC,Zuccato C,Cattaneo E,Néri C,Hayden MR

更新日期：2012-12-01 00:00:00

abstract：:Autophagy and redox biochemistry are two major sub disciplines of cell biology which are both coming to be appreciated for their paramount importance in the etiology of neurodegenerative diseases including Alzheimer's disease (AD). Thus far, however, there has been relatively little exploration of the interface betwee...

journal_title：Neurobiology of disease

authors： Hensley K,Harris-White ME

更新日期：2015-12-01 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：:Prevalent in approximately 20% of the worldwide human population, the rs6265 (also called 'Val66Met') single nucleotide polymorphism (SNP) in the gene for brain-derived neurotrophic factor (BDNF) is a common genetic variant that can alter therapeutic responses in individuals with Parkinson's disease (PD). Possession o...

journal_title：Neurobiology of disease

authors： Mercado NM,Stancati JA,Sortwell CE,Mueller RL,Boezwinkle SA,Duffy MF,Fischer DL,Sandoval IM,Manfredsson FP,Collier TJ,Steece-Collier K

更新日期：2021-01-01 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：: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：:Novel anti-oxidants based on the buckminsterfullerene molecule were explored as neuroprotective agents in cortical cell cultures exposed to excitotoxic and apoptotic injuries. Two polyhydroxylated C60 derivatives, C60(OH)n, n = 12, and C60(OH)nOm, n = 18-20, m = 3-7 hemiketal groups, demonstrated excellent anti-oxidan...

journal_title：Neurobiology of disease

authors： Dugan LL,Gabrielsen JK,Yu SP,Lin TS,Choi DW

更新日期：1996-04-01 00:00:00