Abstract：
:We investigated whether the reduced intramembranous particles (IMP) in the muscle plasma membrane in mdx mice reflects a preferential depletion of a particular size of the IMP. The experiments were performed using the freeze-fracture method to analyze the frequency distribution of the size of IMP, the density of orthogonal array and caveolae in the extensor digitorum longus (EDL) and soleus (SOL) muscles obtained from mdx and control mice. We detected a reduced density of IMP and orthogonal array, and the increased density of caveolae in EDL muscle but not in SOL in the mdx mouse compared with those of the same muscles in control animals. The reduction of IMP was, however, not limited to any specific size of IMP. Our results suggest that the dystrophin associated glycoprotein present in the membrane does not reflect a specific size of IMP. Therefore, our findings indicate that the mechanism of reduced IMP in dystrophinopathy may be different from that of diminished dystrophin binding glycoprotein associated with dystrophin deficiency in Duchenne muscular dystrophy.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Ito H,Yoshimura T,Tsujihata M,Nagataki Sdoi
10.1016/s0022-510x(96)05350-6subject
Has Abstractpub_date
1997-05-29 00:00:00pages
147-51issue
2eissn
0022-510Xissn
1878-5883pii
S0022-510X(96)05350-6journal_volume
148pub_type
杂志文章abstract：INTRODUCTION:Primary lateral sclerosis is a rare neurodegenerative disorder of the upper motor neurons. Diagnostic criteria have changed considerably over the years, and the recent consensus criteria introduced 'probable PLS' for patients with a symptom duration of 2-4 years. The objective of this study is the systemat...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2020.117052
更新日期：2020-10-15 00:00:00
abstract：OBJECTIVE:The prevalence of Parkinson's disease (PD) and costs of healthcare resources for this disease have been increasing in recent years. The objective was to determine the trends in the resource utilization for PD in Germany. METHODS AND PATIENTS:We compared resource utilization in two cohorts of PD patients recr...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,多中心研究
doi：10.1016/j.jns.2010.04.011
更新日期：2010-07-15 00:00:00
abstract：INTRODUCTION:Cerebral infarction secondary to Aspergillus arteritis or septic embolism is an exceptional finding. We present a case of multiple systemic embolism and cerebral infarction resulting from Aspergillus aortitis in an immunocompetent patient. PATIENT:A 65-year-old male with hypertension, hyperglycaemia and m...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2009.04.037
更新日期：2009-09-15 00:00:00
abstract：:In recent years it has become clear that multiple sclerosis (MS) patients benefit from physical exercise as performed in aerobic training but little is known about the effect on functional domains and physiological factors mediating these effects. We studied immunological, endocrine and neurotrophic factors as well as...
journal_title：Journal of the neurological sciences
pub_type： 临床试验,杂志文章
doi：10.1016/j.jns.2004.06.009
更新日期：2004-10-15 00:00:00
abstract：BACKGROUND:Differential diagnosis between idiopathic Parkinson's disease (PD) and multiple system atrophy (MSA) is often difficult in early disease stages. Since MSA is misdiagnosed as PD in more than 20% of the early stages, there is need for methods refining the differentiation of the two disease entities. In PD post...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(00)00279-3
更新日期：2000-04-01 00:00:00
abstract：:As an inhibitor of the immune system and a longevity drug, rapamycin has been suggested as a treatment for Alzheimer's disease, although the underlying mechanisms remain to be clarified. To elucidate the mechanisms, we performed a high-throughput quantitative proteomics analysis and bioinformatics analysis of the chan...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2019.02.022
更新日期：2019-04-15 00:00:00
abstract：:In view of recent reports on lower brainstem dysfunction in Huntington's chorea, we studied respiration during sleep in 12 patients with Huntington's chorea (HC) and in controls. There were no statistically significant differences between patients and controls with respect to apnea periods, respiratory frequency and t...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(88)90174-8
更新日期：1988-03-01 00:00:00
abstract：:Outcome after aSAH depends on several factors, including the severity of the initial event, perioperative medical management, surgical variables, and the incidence of complications. Cerebral vasospasm (CV) is ure to consistently respond to treatment, emphasizing the need for further research into the underlying mechan...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2007.04.046
更新日期：2007-10-15 00:00:00
abstract：:Thirty-six patients with benign intracranial hypertension (BIH) were reviewed. Follow-up was obtained on 33 patients (91%) after a mean period of 7 1/2 years. Precipitating factors were found in 27 patients (75%). On admission, 5 patients had retro-ocular pain, especially on eye movements, a complaint not yet describe...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(79)90200-4
更新日期：1979-02-01 00:00:00
abstract：:The mitochondria have several important functions in the cell. A mitochondrial dysfunction causes an abatement in ATP production, oxidative damage and the induction of apoptosis, all of which are involved in the pathogenesis of numerous disorders. This review focuses on mitochondrial dysfunctions and discusses their c...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2007.01.033
更新日期：2007-06-15 00:00:00
abstract：INTRODUCTION:Cerebrovascular reactivity (CVR) reflects the compensatory dilatory capacity of cerebral arterioles to a dilatory stimulus and is an important mechanism for maintaining constant cerebral blood flow. Many pathological conditions are associated with an impaired CVR thus contributing to a higher risk of cereb...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2006.06.009
更新日期：2006-11-15 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：BACKGROUND:Clinical data across the globe especially in genetic diseases like Huntington's disease (HD) is most helpful when collected using standardized formats. This helps in proper comparison of clinical and genetic data. METHODS:Herein, we report clinical data on 26 genetically confirmed HD patients from 19 Indian...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(01)00593-7
更新日期：2001-09-15 00:00:00
abstract：:There is evidence that visuo-spatial capacity can become overloaded when processing a secondary visual task (Dual Task, DT), as occurs in daily life. Hence, we investigated the influence of the visuo-spatial interference in the identification of emotional facial expressions (EFEs) in early stages of Parkinson's diseas...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2011.09.041
更新日期：2012-02-15 00:00:00
abstract：:We have compared plasma redox status of coenzyme Q-10 in 20 sporadic amyotrophic lateral sclerosis (sALS) patients with those in 20 healthy age/sex-matched controls. A significant increase in the oxidized form of coenzyme Q-10 (sALS=109.3+/-95.2 nM; controls=23.3+/-7.5 nM, P=0.0002) and in the ratio of oxidized form o...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2004.09.030
更新日期：2005-01-15 00:00:00
abstract：:Motor neurone disease (MND) is a useful paradigm for many progressive disabling neurological disorders and serves as a particularly opposite model for the study of patients' responses to progressive and irreversible disability. We studied the progression of disability and the patients' perception of their health in a ...
journal_title：Journal of the neurological sciences
pub_type： 临床试验,杂志文章
doi：10.1016/0022-510x(95)00062-7
更新日期：1995-05-01 00:00:00
abstract：:By observing the effect of 0.08 and 0.02 mg/ml linoleic acid (LA on the electrophoretic mobility of fresh red blood cells derived from the parents of a subject with DMD, it has been shown that all of 16 fathers as well as 15 mothers, consistently differ from normal. In normal subjects, whilst 0.08 mg/ml LA causes incr...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(87)90021-9
更新日期：1987-12-01 00:00:00
abstract：:The kynurenine pathway (KP), the primary route of tryptophan degradation in mammalian cells, consists of many metabolites including kynurenic acid (KYNA), quinolinic acid (QUIN), 3-hydroxykynurenine (3-HK) and picolinic acid (PIC). The former two are neuroactive, while the latter two are molecules with pro-oxidants an...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2012.08.005
更新日期：2012-12-15 00:00:00
abstract：:Room tilt illusion, a misperception that the entire room is tilted, is a rare but nonspecific neurological symptom. We report a patient with Parkinson's disease who developed typical room tilt illusion. Single photon emission computed tomography demonstrated hypoperfusion of the posterior area of the right intrapariet...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2009.08.054
更新日期：2009-12-15 00:00:00
abstract：:Electron-microscopic X-ray microanalysis has been used to compare elemental concentrations in specific organelles in normal and diseased human muscle. An elevated calcium to phosphorus ratio has been found in both myonuclei and interstitial cell nuclei in diseased muscle compared with controls. Preliminary observation...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(77)90129-0
更新日期：1977-09-01 00:00:00
abstract：:Ultrastructural alterations were observed in the sciatic nerve of dystrophic mice. Myelin sheaths were abnormal in shape, abruptly ceased beyond a node of Ranvier, leaving the axon naked. These changes were seen in both afferent and efferent nerve fibres. Apparent embryonal Schwann cells and Schwann cells which were a...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(76)90121-0
更新日期：1976-08-01 00:00:00
abstract：:Alterations in short- and long-latency components of median and tibial somatosensory evoked potentials (SEPs) were studied in patients with lesions in the thalamus and thalamo-cortical radiations. When the lesions were located primarily in the ventro-posterior thalamus, the SEP changes consisted of the following combi...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(86)90169-3
更新日期：1986-12-01 00:00:00
abstract：:Few studies have explored the role of microRNAs (or miRNAs) in Amyotrophic Lateral Sclerosis (ALS) muscle, possibly because of the difficulty in obtaining samples and because this is a rare disease. We measured the expression levels of muscle-specific miRNAs (miRNA-1, miRNA-206, miRNA-133a, miRNA-133b, miRNA-27a) and ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2017.07.008
更新日期：2017-09-15 00:00:00
abstract：:There is significant evidence that the pathogenesis of several neurodegenerative diseases, including Parkinson's disease, Alzheimer's disease, Friedreich's ataxia (FRDA), multiple sclerosis and amyotrophic lateral sclerosis, may involve the generation of reactive oxygen species (ROS) and/or reactive nitrogen species (...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2005.03.012
更新日期：2005-06-15 00:00:00
abstract：:Cathepsin B (CB) activity was measured in aqueous extracts of frozen autopsy specimens of centrum semiovale from three MS patients, five control patients with other neurological diseases, and five normal individuals. Mean activity was significantly increased in the MS tissue compared with controls. The mean activity i...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(95)00039-5
更新日期：1995-07-01 00:00:00
abstract：:The effects of synergist tenotomy have been studied on rat soleus muscles after denervation and after interference with sciatic axoplasmic flow with colchicine. The results suggest that neural, as well as muscular, factors cause compensatory hypertrophy (CH) of soleus. The myogenic factor may be mild depolarization of...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(80)90166-5
更新日期：1980-03-01 00:00:00
abstract：:Refractoriness of the visual evoked potential to pattern-reversal stimulation has been studied using a light-emitting diode stimulator, the effect on a test P100 of a conditioning pattern reversal being determined for conditioning-test intervals of 20 ms, 50 ms and 100 ms. In 20 normal subjects, the latency of the tes...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(86)90064-x
更新日期：1986-03-01 00:00:00
abstract：:Respiratory failure accounts for the majority of deaths in amyotrophic lateral sclerosis (ALS) but only rarely is ALS diagnosed on the basis of respiratory insufficiency. We report four ALS patients presenting with acute respiratory failure. In three patients we have performed EMG needle examination of both hemidiaphr...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(96)00089-5
更新日期：1996-08-01 00:00:00
abstract：:The significance of P300 in investigation of the cognitive changes characteristic of aging and dementia is well established. But some controversies about sensitivity and specificity of P300 latency prolongation in detection of dementia still exist, and its predictive value and specificity in "real" clinical situations...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/0022-510x(95)00093-h
更新日期：1995-08-01 00:00:00
abstract：:The clinical value and practical application of the lumbosacral evoked electrospinogram (Espg) and somatosensory cerebral evoked potentials (SEP) were investigated in 52 patients with conus medullaris or cauda equina lesions. It was shown that the destruction or compression of the conus/cauda equina region by traumati...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(80)90113-6
更新日期：1980-12-01 00:00:00
