Abstract:
:Transgenic mice that express the G93A mutation of human Cu,Zn superoxide dismutase (SOD1(G93A)), found in familial amyotrophic lateral sclerosis (FALS), showed clinical symptoms and histopathological changes of sporadic ALS, including fragmentation of the neuronal Golgi apparatus (GA). The finding of fragmented neuronal GA in asymptomatic mice, months before the onset of paralysis, suggests that the GA is an early target of the pathological processes causing neuronal degeneration. Transgenic mice expressing human SOD1(G93A) have aggregates of mutant protein and ubiquitin in neuronal and glial cytoplasm; they appeared first in the neuropil and later in the perikarya of motor neurons, where they were adjacent to fragmented GA. The aggregates of SOD1(G93A) appeared in neuronal perikarya of asymptomatic mice containing fragmented GA. The numbers of neurons with deposits of SOD1(G93A) and fragmented GA progressively increased with age. Immuno-electron microscopy using colloidal gold showed labeling of ubiquitin and SOD1 over 13 nm thick cytoplasmic filaments. Spinal cord extracts showed a 20-fold increase of SOD1(G93A) in transgenic mice compared to the wild-type protein in controls. The results suggest a causal relationship between the aggregation of mutant SOD1 and ubiquitin, fragmentation of the Golgi apparatus of motor neurons and neurodegeneration.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Stieber A,Gonatas JO,Gonatas NKdoi
10.1016/s0022-510x(99)00300-7subject
Has Abstractpub_date
2000-02-01 00:00:00pages
53-62issue
1eissn
0022-510Xissn
1878-5883pii
S0022-510X(99)00300-7journal_volume
173pub_type
杂志文章abstract::Nitric oxide (*NO)-mediated toxicity has been involved in neurodegenerative diseases, including Parkinson's disease (PD). We have recently reported an increase of about 50% in *NO production rate in PMA-activated polymorphonuclear leukocytes (PMN) from either newly diagnosed or chronically treated PD patients. As humo...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(99)00079-9
更新日期:1999-05-01 00:00:00
abstract::Vascular dementia (VaD) is increasingly recognised to reflect an outmoded concept in that it identifies cases too late for preventive therapy to have an opportunity to prevent the development of dementia and uses a cognitive paradigm inappropriately based on Alzheimer's disease. A replacement is urgently required and ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/s0022-510x(02)00253-8
更新日期:2002-11-15 00:00:00
abstract:INTRODUCTION:The ventral attention network (VAN) is an important mediator of stimulus-driven attention. Multiple cortical areas, such as the middle and inferior frontal gyri, anterior insula, inferior parietal lobule, and temporo-parietal junction have been linked in this processing. However, knowledge of network conne...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2019.116548
更新日期:2020-01-15 00:00:00
abstract::The analysis of the firing intervals of motor units has been suggested as a diagnostic tool in patients with neuromuscular disorders. Part of the increase in number of turns seen in patients with myopathy could be secondary to the decrease in motor unit firing intervals at threshold force of the motor units, as noted ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(87)90077-3
更新日期:1987-03-01 00:00:00
abstract::Coronal images of position emission tomography (PET) by 18F-labelled deoxyglucose reconstructed from interpolated scan in Alzheimer's disease clearly indicated suppression of glucose metabolism in the parietal lobe and lateral part of the temporal lobes, compared to normal controls. The medial part of the temporal lob...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(91)90249-7
更新日期:1991-12-01 00:00:00
abstract::Five French families with Creutzfeldt-Jakob disease (CJD) were found to have either of 2 different point mutations (at codons 178 and 200) in the amyloid precursor gene (PRNP) on chromosome 20. The ancestry of these and other CJD families outside of France suggests that the codon 178 mutation had a northern European o...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(91)90151-v
更新日期:1991-10-01 00:00:00
abstract::There have been recent developments in the pathology of sporadic ALS. A new filamentous neuronal inclusion body in ALS detected by immunohistochemical localisation of the protein ubiquitin has been characterised at the light microscopic and ultrastructural level and appears specific for the disease. The molecular comp...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/0022-510x(94)90175-9
更新日期:1994-07-01 00:00:00
abstract:BACKGROUND:The Oxfordshire Community Stroke Project (OCSP) classification is a simple tool to categorize clinical stroke syndromes. We compared the outcomes of stroke patients after intravenous thrombolysis stratified by the baseline National Institutes of Health Stroke Scale (NIHSS) score or by the OCSP classification...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2012.10.003
更新日期:2013-01-15 00:00:00
abstract::The expression of class II antigen was studied in sural nerve biopsies from patients with peripheral neuropathies. These included patients with chronic demyelinating polyradiculoneuropathy (CIDP), non-immune mediated neuropathies of diverse etiologies and controls without evidence of neuropathy. The major finding in C...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(91)90065-f
更新日期:1991-04-01 00:00:00
abstract::In an attempt to obtain insight into the pathogenesis of proteolipid apoprotein (PLP)-induced experimental allergic encephalomyelitis (EAE) in Lewis rats (Yamamura et al. 1986), PLP-sensitized lymph node cells or spleen cells were passively transferred into normal or irradiated (400 rad) recipients after incubation wi...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(86)90174-7
更新日期:1986-12-01 00:00:00
abstract::Adoptive transfer of experimental allergic encephalomyelitis (EAE) was achieved in Lewis rats using spleen cells incubated in vitro with various lectins. When stimulated with concanavalin A (Con A), its derivative succinyl-Con A, Lens culinaris or Pisum sativum agglutinin, spleen cells obtained from rats immunized wit...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(84)90014-5
更新日期:1984-11-01 00:00:00
abstract::Fast and slow components of anterograde axoplasmic transport have been studied in the sensory fibres of the vagus nerve of alloxan-diabetic rabbits and age-matched controls by incorporation of tritiated leucine into nodose ganglion cells. The diabetic rabbits were maintained for 2 months with blood glucose levels in t...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(80)90089-1
更新日期:1980-09-01 00:00:00
abstract:BACKGROUND:The timed-up-and-go test (TUG) is a popular test for mobility, but it only measures the total time to complete tasks. Recent advances in smartphone technology enables measuring respective times of 6 components in the test (Stand, Go, Turn1, Come, Turns and Sit). We examined availability of these data for ass...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2019.01.023
更新日期:2019-03-15 00:00:00
abstract::In addition to neuritic changes and amyloid deposits, neuronal and glial cell apoptosis is an important pathological feature of Alzheimer's disease (AD). Several factors have been postulated as causes or triggers of cellular apoptotic change. This study focused on a quantifiable relationship between phosphorylation si...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(02)00410-0
更新日期:2003-04-15 00:00:00
abstract::Interaction of the orexinergic (ORXergic) neuronal system with the excitatory (glutamate, l-Glu) or the inhibitory (GABA) neurosignaling complexes evokes major homeostatic physiological events. In this study, effects of the two ORXergic neuropeptides (ORX-A/B) on their receptor (ORX-2R) expression changes were correla...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2015.02.030
更新日期:2015-04-15 00:00:00
abstract:BACKGROUND:Olfactory identification impairment in amnestic mild cognitive impairment (aMCI) patients is well documented and considered to be caused by underlying Alzheimer's disease (AD) pathology, contrasting with less clear evidence in non-amnestic MCI (naMCI). The aim was to (a) compare the degree of olfactory ident...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2015.01.014
更新日期:2015-02-15 00:00:00
abstract:BACKGROUND:Restless legs syndrome (RLS) is characterized by the urge to move the legs accompanied by movement-responsive, abnormal sensations, which worsen at rest and night. We investigated the distribution of sensory symptoms and clinical correlations in patients with RLS and its variants. METHODS:Eighty-nine patien...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2019.116519
更新日期:2019-12-15 00:00:00
abstract::Recent studies have demonstrated that cytokines play an important role in the pathogenesis of intracranial aneurysms (IAs). Interleukin-6 (IL-6) is an important proinflammatory cytokine. In our study, we investigated the association of genetic variants within the gene encoding interleukin-6-572G/C (IL-6-572G/C) with I...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2011.03.036
更新日期:2011-07-15 00:00:00
abstract:BACKGROUND:There is little information about intracranial atherosclerotic disease (ICAD) and extracranial atherosclerotic disease (ECAD) in patients with acute spontaneous intracerebral hemorrhage (ICH). The purpose of the present study was to elucidate the prevalence of and the factors that correlate with ICAD and ECA...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2013.06.031
更新日期:2013-09-15 00:00:00
abstract:OBJECTIVE:Aneurysm growth is a risk factor for rupture, however the detailed mechanism remains unclear. The present study was performed to identify whether hemodynamic insult could prompt small unruptured aneurysms to grow. METHODS:Six pairs of unruptured small (<5 mm) cerebral aneurysms from patients followed with lo...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2020.116798
更新日期:2020-06-15 00:00:00
abstract::The notion that there is an association between essential tremor (ET) and higher ethanol consumption has crept into the literature; however, the data are limited and conflicted. A total of 354 ET cases and 370 matched controls were enrolled in a clinical-epidemiological study. Average current daily ethanol consumption...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2014.09.042
更新日期:2014-12-15 00:00:00
abstract::MabQ113 is a monoclonal antibody raised against rat cerebellum which selectively strains Purkinje cells. Likewise, in mabQ113-immunoperoxidase stained sections of human cerebellum, deposits of reaction product are found only in the Purkinje cells. The dendritic arborizations, cell body, and axonal processes are immuno...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(85)90166-2
更新日期:1985-10-01 00:00:00
abstract::We report eight patients with adult-onset subacute sclerosing panencephalitis (SSPE), of which, four were treated with oral isoprinosine and four with intraventricular alpha-interferon plus oral isoprinosine. One of the four patients treated with oral isoprinosine died within two months, and the disease progressed in ...
journal_title:Journal of the neurological sciences
pub_type: 临床试验,杂志文章
doi:10.1016/s0022-510x(98)00302-5
更新日期:1999-01-01 00:00:00
abstract::Five males in one kindred suffered from intermittent ataxia and one female may have been more mildly affected. The pattern of inheritance strongly suggests X-linkage. Cerebral pathology in one case had some features of Leigh's disease. A defect in pyruvate metabolism was found in two cases. Acetazolamide gave a tempor...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(84)90059-5
更新日期:1984-04-01 00:00:00
abstract:OBJECTIVES:There is insufficient data on the efficacy of intravenous immunoglobulins (IVIg) as maintenance treatment in myasthenia gravis (MG). We therefore examined response to maintenance IVIg therapy in a cohort of MG patients. METHODS:We reviewed all MG patient files treated with IVIg in our neuro-immunology clini...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2013.10.043
更新日期:2014-03-15 00:00:00
abstract::We measured the magnetization transfer (MT) ratios in white matter lesions of Binswanger's disease (BD) and compared them with BD and with similar-appearing changes in non-demented elderly subjects and cerebral infarction. Four subject groups were studied: 30 patients with BD and periventricular hyperintensity (PVH) o...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(99)00122-7
更新日期:1999-07-01 00:00:00
abstract:OBJECTIVES:To characterize the clinical, demographic and epidemiological features of MS patients from the only specialised MS centre in Iraq. METHODS:Data for consecutive Iraqi MS patients attending the Baghdad Multidiscipline MS Clinic between 2000 and 2002 who fulfilled Poser et al. criteria for clinically definite ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2005.03.033
更新日期:2005-07-15 00:00:00
abstract:BACKGROUND:Contactin-associated protein 2 (Caspr2) antibody is a neuronal surface antibody (NSAb) capable of causing disorders involving central and peripheral nervous systems (PNS). Thymoma can be found in patients with Caspr2 antibodies and is most frequently associated with PNS symptoms. Myasthenia gravis can be fou...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2014.03.043
更新日期:2014-06-15 00:00:00
abstract::A 52-year-old Japanese woman complaining of horizontal double vision for 10 days was admitted to our hospital. Neurological examination revealed left abducent nerve palsy and muscle swelling in her thighs. Brain MRI showed obstruction in the spinal fluid space of the left Dorello's canal, which transmits a portion of ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2014.05.055
更新日期:2014-08-15 00:00:00
abstract::Water-suppressed proton magnetic resonance spectroscopy which has recently shown diagnostic potential in human stroke victims was performed in two patients who had suffered a hemispheric transient ischemic attack. Localized spectra were targeted from routine MR images. In one patient lactate could be observed 8 weeks ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(92)90185-n
更新日期:1992-03-01 00:00:00