Abstract:
:A host protein encoded by the gene specifying the scrapie amyloid precursor affects pathogenesis of the transmissible spongiform encephalopathies: Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker's syndrome (GSS), and kuru in man, and scrapie in animals. We found a mutation in this gene of two patients with CJD from one family and a second mutation in the same gene in three patients with GSS from another family. The mutation in two related familial CJD patients changed glutamine in position 200 tolysine. This mutation was absent in other individuals including unrelated patients with familial CJD, sporadic CJD, and GSS. The other mutation in three GSS patients changed proline in position 102 to leucine, the same mutation described recently in some GSS families. We did not find it in six unaffected relatives of the GSS patients or in other individuals including sporadic and familial CJD patients. A rare insertion described earlier in one CJD family was also absent in all tested individuals.
journal_name
Exp Neuroljournal_title
Experimental neurologyauthors
Goldgaber D,Goldfarb LG,Brown P,Asher DM,Brown WT,Lin S,Teener JW,Feinstone SM,Rubenstein R,Kascsak RJdoi
10.1016/0014-4886(89)90095-2subject
Has Abstract,Author List Incompletepub_date
1989-11-01 00:00:00pages
204-6issue
2eissn
0014-4886issn
1090-2430journal_volume
106pub_type
杂志文章abstract::The presence of the alpha2macroglobulin receptor/low density lipoprotein receptor-related protein (alpha2Mr/LRP) and its ligands alpha2macroglobulin (alpha2M), apoliprotein E, and plasminogen activators was detected in senile plaques of Alzheimer's disease (AD). To explore a possible role of alpha2M in neurodegenerati...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1006/exnr.1998.6978
更新日期:1999-02-01 00:00:00
abstract::Chronic administration of cocaine (10 mg/kg, i.p., every 12 h for 10 consecutive days) produced a large decrease in tyrosine hydroxylase-staining axons and terminal boutons in the caudate nucleus in rats when examined 60 days after the final cocaine injection. This effect was quantitated using the Leitz data acquisiti...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/0014-4886(86)90252-9
更新日期:1986-12-01 00:00:00
abstract::Ciliary neurotrophic factor (CNTF) and brain-derived neurotrophic factor (BDNF) have been shown both in vitro and in vivo to support the survival and differentiation of developing chick or rat motoneurons. To explore the potential use of these and other neurotrophic factors as therapeutic agents in human motoneuron di...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1006/exnr.1994.1198
更新日期:1994-12-01 00:00:00
abstract::The roles of activated NF-kappaB subunits in the CNS remain to be discerned. Members of this family of transcription factors are essential to diverse physiological processes and can be activated by pathogens, stress, pharmacological agents, and trauma. We are particularly interested in long-term NF-kappaB activation a...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1006/exnr.2002.7967
更新日期:2002-08-01 00:00:00
abstract::Traumatic brain injury (TBI) causes persistent neurologic deficits. Current therapies, predominantly focused upon cortical and hippocampal cellular survival, have limited benefit on cognitive outcomes. Striatal damage is associated with deficits in executive function, learning, and memory. Dopamine and cAMP regulated ...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/j.expneurol.2011.02.013
更新日期:2011-06-01 00:00:00
abstract::Prostaglandin and thromboxane levels increase in mammalian brain during seizures, but whether a metabolite of arachidonic acid has a role in induction, or is merely a by-product, of seizures is still not clear. It has been shown that nonsteroidal anti-inflammatory drugs block arachidonic acid metabolism through inhibi...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/0014-4886(87)90080-x
更新日期:1987-10-01 00:00:00
abstract::Laser Doppler flowmetry was utilized to examine blood flow in sciatic nerves of barbiturate-anesthetized and of unanesthetized decerebrate rats, in response to hypotension induced by graded exsanguination or by graded clamping of the descending aorta. Continuous laser Doppler flowmetry signals decreased linearly with ...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/0014-4886(85)90047-0
更新日期:1985-10-01 00:00:00
abstract::Posttraumatic stress disorder (PTSD), chronic psychological stress, and major depressive disorder have been found to be associated with a significant decrease in glial fibrillary acidic protein (GFAP) immunoreactivity in the hippocampus of rodents. Cotinine is an alkaloid that prevents memory impairment, depressive-li...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/j.expneurol.2017.06.016
更新日期:2017-09-01 00:00:00
abstract::Overexpression of nerve growth factor (NGF) using adenoviruses (Adts) after spinal cord injury induces extensive regeneration and sprouting of calcitonin-gene-related peptide immunoreactive (CGRP-IR) fibers, whereas overexpression of cell adhesion molecules (CAMs) has no effect on the normal distribution of these fibe...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/j.expneurol.2006.06.001
更新日期:2006-11-01 00:00:00
abstract::CNS damage often results in demyelination of spared axons due to oligodendroglial cell death and dysfunction near the injury site. Although new oligodendroglia are generated following CNS injury and disease, the process of remyelination is typically incomplete resulting in long-term functional deficits. Chondroitin su...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/j.expneurol.2013.04.003
更新日期:2013-09-01 00:00:00
abstract::Peripheral nerves have the unique capability to regenerate after injury. Insights into regeneration of peripheral nerves after injury may have implications for neurodegenerative diseases of the nervous system. In this study, we analyzed the expression and function of desmoplakin in peripheral nerve regeneration. Desmo...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/j.expneurol.2014.12.005
更新日期:2015-02-01 00:00:00
abstract::Damage to the medial frontal cortex in rats results in a learning deficit on a reinforced alternation task. The rate of recovery from this deficit was accelerated by transplantation of either adult or embryonic frontal cortex, provided that a delay was introduced between injury and transplantation. The rates of recove...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/0014-4886(86)90241-4
更新日期:1986-12-01 00:00:00
abstract::Urethane-induced cortical slow wave activity (SWA) spreads into the basal ganglia in dopamine (DA)-depleted rat models of Parkinson's disease (PD). During physiological sleep, SWA is powerfully expressed at the beginning of night and progressively reduced during sleep-time reflecting the sleep need. However, its under...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/j.expneurol.2017.12.004
更新日期:2018-03-01 00:00:00
abstract::Alpha-tyrosinated tubulin is a cytoskeletal protein that is involved in axonal growth and is considered a marker of neuronal plasticity in adult mammals. In adult rats, unilateral ablation of the left facial sensorimotor cortical areas induces degeneration of corticotrigeminal projections and marked denervation of the...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/j.expneurol.2008.05.010
更新日期:2008-09-01 00:00:00
abstract::Cannabis abuse may precipitate the onset of schizophrenia and a dysfunction of the endocannabinoid system may be involved in the pathology of schizophrenia. Nevertheless, only few studies have addressed the neurobiological consequences of cannabis abuse for the development and course of schizophrenia. We measured the ...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/j.expneurol.2007.02.004
更新日期:2007-05-01 00:00:00
abstract::The piriform cortex is recognized to play critical roles in focal ictogenesis, both in animal models and in humans. We review here the contribution of in vitro studies performed on rodent brain tissue that were aimed at understanding the ictogenic properties of the piriform cortex and the contiguous olfactory areas. D...
journal_title:Experimental neurology
pub_type: 杂志文章,评审
doi:10.1016/j.expneurol.2019.113014
更新日期:2019-11-01 00:00:00
abstract::It has been reported that the severe cerebral edema produced in experimental animals by Clostridium perfringens (Cl p) type D epsilon toxin can be prevented by prior treatment with its precursor prototoxin due to competitive binding to endothelial cells (ECs) at the blood-brain barrier (BBB). In this study we investig...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1006/exnr.2001.7652
更新日期:2001-05-01 00:00:00
abstract::beta-N-methylamino-l-alanine (BMAA) is a non-protein amino acid implicated in the neurodegenerative disease amyotrophic lateral sclerosis/Parkinson-dementia complex (ALS/PDC) on Guam. BMAA has recently been discovered in the brains of Alzheimer's patients in Canada and is produced by various species of cyanobacteria a...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/j.expneurol.2009.04.002
更新日期:2009-06-01 00:00:00
abstract::Brain-derived neurotrophic factor (BDNF) plays an essential role in sensory neuronal activation in response to visceral inflammation. Here we report that BDNF up-regulation in the primary afferent neurons in the dorsal root ganglia (DRG) in a rat model of colitis is mediated by the activation of endogenous extracellul...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/j.expneurol.2012.08.007
更新日期:2012-12-01 00:00:00
abstract::The beta-amyloid precursor protein (betaAPP) is found in skeletal muscle localized to the base of the postsynaptic folds of the neuromuscular junction; yet here, as well as in neurons, its function remains enigmatic. Here we report that the motor nerve-derived trophic factor neuregulin-1 (NRG1) regulates both steady-s...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/s0014-4886(03)00031-1
更新日期:2003-06-01 00:00:00
abstract::Monosialoganglioside GM1 prevents excitatory amino acid (EAA)-related neuronal death in cultured central nervous system (CNS) neurons and reduces the severity of acute brain damage in different experimental models of cerebral ischemia. Using a model of brain damage induced by intracerebroventricular administration of ...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/0014-4886(91)90019-9
更新日期:1991-09-01 00:00:00
abstract::Previous studies have suggested that defective high-affinity glutamate uptake, due mainly to a major loss of the astroglial-specific GLT-1 glutamate transporter, underlies the selective motoneuron degeneration observed in sporadic ALS (24, 28). If a defect in glutamate transport underlies the pathogenesis of sporadic ...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1006/exnr.1996.0198
更新日期:1996-12-01 00:00:00
abstract::Transplantation of genetically engineered cells can provide sustained focal delivery of naturally occurring molecules, including neurotransmitters and growth factors. We have engineered immortalized mouse cortical neurons and glia to deliver GABA by driving GAD(65) expression. Engineered cell lines showed GAD(65) mRNA...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1006/exnr.1999.7305
更新日期:2000-02-01 00:00:00
abstract::Given the prevalence and societal impact of autism spectrum disorders (ASD), there is an urgent need to develop innovative preventative strategies and treatments to reduce the alarming number of cases and improve core symptoms for afflicted individuals. Translational efforts between clinical and preclinical research a...
journal_title:Experimental neurology
pub_type: 杂志文章,评审
doi:10.1016/j.expneurol.2017.07.021
更新日期:2018-01-01 00:00:00
abstract::Immune rejection of transplanted material is a potential complication of organ donation. In response to tissue transplantation, immune rejection has two components: a host defense directed against the grafted tissue and an immune response from the grafted tissue against the host (graft vs host disease). To treat immun...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/s0014-4886(03)00128-6
更新日期:2003-08-01 00:00:00
abstract::Although transgenic expression of oncogenes typically leads to tumorigenesis, oncogene expression directed to the rod photoreceptors leads to cell death without tumor formation. To evaluate the cellular and functional changes induced in cone photoreceptors by an oncogene, the Mas1 protooncogene was targeted to the con...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1006/exnr.2000.7370
更新日期:2000-05-01 00:00:00
abstract::The anticonvulsant long-term efficacy of diazepam was studied in amygdala-kindled rats. The drug was administered three times daily at doses of 5 mg/kg i.p. for 2 weeks in fully kindled animals. The severity of the kindled seizures was markedly reduced throughout the period of treatment, while tolerance developed to t...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/0014-4886(85)90026-3
更新日期:1985-11-01 00:00:00
abstract::Unit activity related to a go/no-go visual discrimination task was studied in four rhesus monkeys. We recorded 272 task-related cells from frontal cortex in a region extending from the midprincipal sulcus to the central sulcus, and medially to the cingulate sulcus. Units located in anterior regions (dorsolateral prefr...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/0014-4886(87)90213-5
更新日期:1987-06-01 00:00:00
abstract::Synaptic reorganization of mossy fibers following kainic acid (KA) administration has been reported to contribute to the formation of recurrent excitatory circuits, resulting in an epileptogenic state. It is unclear, however, whether KA-induced mossy fiber sprouting results from neuronal cell loss or the seizure activ...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1006/exnr.1999.7251
更新日期:2000-01-01 00:00:00
abstract::The Alzheimer's disease-related peptide beta(1-40) amyloid self-associates to form fibrils exhibiting a morphology characteristic of amyloidogenic proteins. The mechanism of this fibrillization process has yet to be fully elucidated. In this study we have immobilized the beta(1-40) amyloid to flat gold surfaces using ...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1006/exnr.1999.7114
更新日期:1999-08-01 00:00:00