Abstract:
:Mutations in fused in sarcoma and/or translocated in liposarcoma (FUS, TLS or FUS) are linked to familial cases of amyotrophic lateral sclerosis (ALS). Mutant FUS selectively accumulates into discrete cytosolic structures known as stress granules under various stress conditions. In addition, mutant FUS expression can alter the dynamics and morphology of stress granules. Although the link between mutant FUS and stress granules is well established, the mechanisms modulating stress granule formation and disassembly in the context of ALS are poorly understood. In this issue of Neurobiology of Aging, Ryu et al. uncover the impact of autophagy on the potential toxicity of mutant FUS-positive stress granules. The authors provide evidence indicating that enhanced autophagy activity reduces the number of stress granules, which in the case of cells containing mutant FUS-positive stress granules, is neuroprotective. Overall, this study identifies an intersection between the proteostasis network and alterations in RNA metabolism in ALS through the dynamic assembly and disassembly of stress granules.
journal_name
Neurobiol Agingjournal_title
Neurobiology of agingauthors
Matus S,Bosco DA,Hetz Cdoi
10.1016/j.neurobiolaging.2014.08.019subject
Has Abstractpub_date
2014-12-01 00:00:00pages
2832-2835issue
12eissn
0197-4580issn
1558-1497pii
S0197-4580(14)00532-6journal_volume
35pub_type
杂志文章abstract::Decline in fluid abilities in normal aging is associated with increased white matter lesions, measured on T1-weighted images as white matter signal abnormalities (WMSAs). WMSAs are particularly evident in hypertensive older adults, suggesting vascular involvement. However, because hypertension is assessed systemically...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/j.neurobiolaging.2019.08.004
更新日期:2019-12-01 00:00:00
abstract::Age-related changes in resting levels of the free intracellular calcium concentration ([Ca2+]i) as well as alterations of the rise in [Ca2+]i following depolarization have been investigated in acutely isolated brain cells of various regions of the rat brain. Characterization of the Ca2+ responses following KCl depolar...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/0197-4580(96)84400-9
更新日期:1996-07-01 00:00:00
abstract::Insomnia, daytime sleepiness, and nocturnal wandering, so common in the elderly, are caused largely by two specific pathophysiologic processes. Sleep apnea is a condition where respiration pauses during sleep, leading to arousal. Sleep apnea is due either to obstruction in the throat or failure of the central respirat...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/0197-4580(82)90021-5
更新日期:1982-01-01 00:00:00
abstract::Large repeat expansions in the C9orf72 gene were recently reported to be a major cause of familial amyotrophic lateral sclerosis and frontotemporal dementia. Given some of the clinical and pathologic overlap between these 2 diseases and Parkinson's disease, we sought to evaluate the presence of these expansions in a c...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/j.neurobiolaging.2012.11.025
更新日期:2013-06-01 00:00:00
abstract::Mutants of presenilin 1 (PS1) increase neuronal cell death causing autosomal-dominant familial Alzheimer's disease (FAD). Recent literature shows that treatment of neuronal cultures with low concentrations of trypsin, a member of the serine family of proteases, protects neurons from toxic insults by binding to the pro...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/j.neurobiolaging.2016.02.028
更新日期:2016-06-01 00:00:00
abstract::In a prospective study of dementia in Flanders (Belgium), we observed a substantial fraction of early-onset dementia patients who did not fulfill the criteria for a specific dementia subtype, leaving the patients without a precise clinical diagnosis. We selected 211 of these patients for genetic testing of causal gene...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/j.neurobiolaging.2018.04.015
更新日期:2018-09-01 00:00:00
abstract::A group of alterations in the structure of cortical microvessels is described in the brain tissue of neuropathologically confirmed patients with Alzheimer's disease. These changes include irregular thickening of the vessel walls, infiltration with amyloid and the serum P component of amyloid, loss of the perivascular ...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/s0197-4580(88)80014-9
更新日期:1988-01-01 00:00:00
abstract::Patients with Mild Cognitive Impairment (MCI), exhibiting both working memory and olfactory deficits are likely to progress to Alzheimer's disease (AD). Targeting this pre-clinical AD population with disease modifying agents or cognitive enhancers represents the best strategy for halting or delaying the impact of this...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/j.neurobiolaging.2007.11.018
更新日期:2009-09-01 00:00:00
abstract::In the axon terminals of the locus coeruleus (LC) neurons, a high level of axonal branching was occurred in the middle-aged brain, and the increased branching was maintained in the aged brain. In the present study, we hypothesized that neurotrophic support is necessary for the morphological age-related changes seen in...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/S0197-4580(03)00093-9
更新日期:2004-03-01 00:00:00
abstract::Neurofibrillary degeneration in the nucleus basalis and a loss of its cortical cholinergic projections are prominent components of the neuropathology in Alzheimer's disease (AD). The AD brain is also associated with a degeneration of the noradrenergic projections arising from the nucleus locus coeruleus (LC), but the ...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/j.neurobiolaging.2006.02.007
更新日期:2007-03-01 00:00:00
abstract::Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are adult-onset neurodegenerative diseases with overlapping clinical characteristics. They share common genetic causes and pathologic hallmarks such as TDP-43 neuronal accumulations. Recently, exome analysis identified mutations in matrin...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/j.neurobiolaging.2014.07.016
更新日期:2014-12-01 00:00:00
abstract::Abnormal elevations in ammonia have been implicated in the pathogenesis of Alzheimer's disease. However, the biochemical mechanism(s) leading to increased ammonia in Alzheimer's disease have not yet been identified. A potential source of increased ammonia production is adenosine monophosphate (AMP) deaminase, an impor...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/s0197-4580(98)00083-9
更新日期:1998-09-01 00:00:00
abstract::Transcranial electrical stimulation (tES) is a potentially viable tool for boosting cognitive performance in aging. However, most knowledge on tES effects is based on studies involving young adults. Here, we applied tES (transcranial random noise stimulation [tRNS] as an effective stimulation and anodal transcranial d...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/j.neurobiolaging.2019.07.009
更新日期:2019-10-01 00:00:00
abstract::Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disease characterized by loss of motor neurons. We have recently identified SOD1 and FUS mutations as the most common causes in a consecutive series of 111 familial ALS pedigrees in Japan. To reveal possible genetic causes for the remaining 51 pat...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/j.neurobiolaging.2017.01.004
更新日期:2017-05-01 00:00:00
abstract::Altered glutamate transport and aberrant EAAT1 expression were shown in Alzheimer's disease (AD) brains. It is presently unknown whether these modifications are a consequence of neurodegeneration or play a pathogenetic role. However, recent findings of decreased glutamate uptake, EAAT1 protein and mRNA in AD platelets...
journal_title:Neurobiology of aging
pub_type: 临床试验,杂志文章
doi:10.1016/j.neurobiolaging.2004.07.007
更新日期:2005-06-01 00:00:00
abstract::The aim of this study was to compare the patterns of grey and white matter atrophy on MRI in autopsy confirmed progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD), and to determine whether the patterns vary depending on the clinical syndrome. Voxel-based morphometry was used to compare patterns of...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/j.neurobiolaging.2006.09.019
更新日期:2008-02-01 00:00:00
abstract::Alertness is fundamental for the efficiency of information processing. A person's level of alertness refers to the system's state of general responsiveness and can be temporarily increased by presenting a neutral warning cue shortly before an event occurs (Posner and Petersen, 1990). However, effects of alerts on subs...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/j.neurobiolaging.2019.03.017
更新日期:2019-07-01 00:00:00
abstract::The review by Harbaugh describes new methods for delivering drugs to the diseases and damaged central nervous system. The potential of these methods, infusible pumps, implantable polymers, and neural transplants, is truly extraordinary. One can anticipate that many neurological disorders may be treated by these method...
journal_title:Neurobiology of aging
pub_type: 临床试验,杂志文章
doi:10.1016/0197-4580(89)90166-8
更新日期:1989-09-01 00:00:00
abstract::Sporadic Alzheimer's disease (AD) appears to be the consequence of the interaction between combinations of genes and environmental factors. Binding with the transporter associated with antigen processing (TAP) is thought to be the main way in which herpes simplex virus type 1 (HSV-1) evades immune surveillance. Severa...
journal_title:Neurobiology of aging
pub_type: 杂志文章,多中心研究
doi:10.1016/j.neurobiolaging.2006.02.011
更新日期:2007-04-01 00:00:00
abstract::Accumulating evidence indicates that white matter degeneration contributes to the neural disconnections that underlie Alzheimer's disease pathophysiology. Although this white matter degeneration is partly attributable to axonopathy associated with neuronal degeneration, amyloid β (Aβ) protein-mediated damage to oligod...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/j.neurobiolaging.2010.05.007
更新日期:2012-03-01 00:00:00
abstract::Dualistic effects of acetylcholine-esterase inhibitors on neuronal as well as vasoregulative function have been debated. This study investigated for the first time effects of medication on both components. Visually evoked potentials and resultant hemodynamic responses were assessed in Alzheimer patients (n=31) without...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/j.neurobiolaging.2008.02.017
更新日期:2009-12-01 00:00:00
abstract::Recent reports have suggested that beta-amyloid (A beta) species of variable length C-termini are differentially deposited within early and late-stage plaques and the cerebrovasculature. Specifically, longer C-terminal length A beta 42/3 fragments (i.e., A beta forms extending to residues 42 and/or 43) are thought to ...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/0197-4580(96)00062-0
更新日期:1996-07-01 00:00:00
abstract::Glycogen synthase kinase-3β (GSK3B) and cyclin-dependent kinase 5 (CDK5) are the 2 major protein kinases involved in abnormal phosphorylation of tau. To determine their potential role in the pathogenesis of Parkinson's disease (PD) we analyzed 2 functional single nucleotide polymorphisms (SNPs) of GSK3B (rs334558 and ...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/j.neurobiolaging.2010.10.016
更新日期:2012-07-01 00:00:00
abstract::The amyloid beta-peptide (A beta)-associated free radical oxidative stress model for neuronal death in Alzheimer's disease (AD) brain predicts that neuronal protein oxidation is a consequence of A beta-associated free radicals [8]. In this study we have used both in vitro and in vivo models of beta-amyloid (A beta) to...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/s0197-4580(99)00056-1
更新日期:1999-05-01 00:00:00
abstract::After a decade of intense study of cholinergic therapies for Alzheimer's disease, three conditions in this field are apparent: 1) The potential that cholinergic agents will ameliorate the memory dysfunction of Alzheimer patients (as 1-dopa benefits Parkinson patients) is still a stimulus for research. 2) Cholinergic n...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/s0197-4580(89)80017-x
更新日期:1989-01-01 00:00:00
abstract::Mild cognitive impairment (MCI) represents a heterogeneous group of cognitive disturbances at high risk of dementia. The amnestic subtype (aMCI) might be a prodromal state of Alzheimer's disease (AD). The aim of this study is the identification, by proton magnetic resonance spectroscopy (1H MRS), of modifications in b...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/j.neurobiolaging.2005.05.002
更新日期:2006-07-01 00:00:00
abstract::Amnestic mild cognitive impairment (aMCI) is accompanied by the accelerated cognitive decline and rapid brain degeneration with aging. However, the age-related alterations of the topological organization of the brain connectome in aMCI patients remained largely unknown. In this study, we constructed the brain structur...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/j.neurobiolaging.2017.08.005
更新日期:2017-11-01 00:00:00
abstract::Alzheimer's disease is a progressive neurodegenerative disorder that is characterized by extensive deposition of fibrillar amyloid-β (Aβ) in the brain. Previously, myelin basic protein (MBP) was identified to be a potent inhibitor to Aβ fibril formation, and this inhibitory activity was localized to the N-terminal res...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/j.neurobiolaging.2014.10.006
更新日期:2015-02-01 00:00:00
abstract::Amyloid plaques are a characteristic feature in Alzheimer's disease (AD). A novel non-toxic contrast agent is presented, Gd-DTPA-K6Abeta1-30, which is homologous to Abeta, and allows plaque detection in vivo. microMRI was performed on AD model mice and controls prior to and following intracarotid injection with Gd-DTP...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/j.neurobiolaging.2006.12.018
更新日期:2008-06-01 00:00:00
abstract::The goals were to investigate implicit learning in mild cognitive impairment (MCI), and to determine the relations of implicit learning systems to apolipoprotein E (ApoE) genotype in healthy controls. Elderly controls grouped by ApoE status (ApoE-e4 allele carriers versus ApoE-e4 allele non-carriers) and MCI patients ...
journal_title:Neurobiology of aging
pub_type: 杂志文章
doi:10.1016/j.neurobiolaging.2006.04.004
更新日期:2007-06-01 00:00:00