Abstract:
:Primary cultures prepared from human muscle biopsies were examined for the presence and distribution of acetylcholine receptors, as measured by the binding of 125iodine-labelled alpha-bungarotoxin. The toxin bound to the human muscle cultures with a similar time dependence and specificity as found in muscle cultures from other species. The amount of toxin bound was lower than that obtained for neonatal mouse muscle under similar conditions. The distribution of receptors was similar in cultures derived from the muscles of patients with a variety of neuromuscular disorders. The toxin was located along the myotubes in a fairly even distribution; however, variations in labelling along a single myotube were observed, as well as variations between different myotubes in the same culture. Occasionally, the toxin also bound to other cells, which may have been mononucleated. Cultures prepared from patients with Duchenne muscular dystrophy produced multi-layered cell clusters, instead of the usual monolayer of cells. Within these clusters, only the myotubes bound alpha-bungarotoxin.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Franklin GI,Yasin R,Hughes BP,Thompson EJdoi
10.1016/0022-510x(80)90085-4subject
Has Abstractpub_date
1980-09-01 00:00:00pages
317-27issue
3eissn
0022-510Xissn
1878-5883pii
0022-510X(80)90085-4journal_volume
47pub_type
杂志文章abstract:BACKGROUND:Myasthenia gravis (MG) is the autoimmune disorder in which the thymus is considered the pathogenic organ. Thymectomy (TE) is a therapeutic option for MG and often ameliorates clinical symptoms. METHODS:We evaluated clinical features and outcomes after TE in patients without thymoma and the influence of TE w...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2015.08.029
更新日期:2015-11-15 00:00:00
abstract::The effects of long-term exposure to aluminium on the development of Alzheimer-type neuropathological changes have been studied post-mortem in patients with chronic renal failure who did not have dialysis encephalopathy. Administration of aluminium-containing phosphate binding compounds appears to be a major factor in...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(92)90291-r
更新日期:1992-02-01 00:00:00
abstract::Formation of protein aggregation is considered a hallmark feature of various neurological diseases. Amyotrophic lateral sclerosis is one such devastating neurodegenerative disorder characterized by mutation in Cu/Zn superoxide dismutase protein (SOD1). In our study, we contemplated the most aggregated and pathogenic m...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2019.116425
更新日期:2019-10-15 00:00:00
abstract::On September 28, 1994, the investigators of the Asymptomatic Carotid Atherosclerosis Study (ACAS) reported the interim results of a randomized controlled clinical trial of carotid endarterectomy in patients who have asymptomatic carotid stenosis of greater than 60% reduction in diameter. In addition to aspirin and agg...
journal_title:Journal of the neurological sciences
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.1016/0022-510x(95)00010-y
更新日期:1995-03-01 00:00:00
abstract:INTRODUCTION:Magnetization transfer ratio (MTR) histogram analysis can be used as a method for quantifying overall disease burden in MS. We studied correlations between MTR histogram and clinical parameters in MS subgroups. Contrary to earlier studies we placed special emphasis on the lower MTR range, to explore the ef...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(01)00431-2
更新日期:2001-03-01 00:00:00
abstract:BACKGROUND AND PURPOSE:Although elevated fasting blood glucose (FBG) at admission is associated with poor outcome in patients with ischemic and hemorrhagic stroke, it has not been investigated in patients with cerebral venous thrombosis (CVT). We aimed to determine the correlation between elevated FBG and severity and ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2020.117017
更新日期:2020-10-15 00:00:00
abstract::Benign familial neonatal convulsions (BFNC) are a rare autosomal dominant inherited epilepsy syndrome. Two voltage-gated potassium channel genes, KCNQ2 on chromosome 20q13.3 and KCNQ3 on chromosome 8q24, have been identified as the genes responsible for benign familial neonatal convulsions. By linkage analysis and mut...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2004.03.001
更新日期:2004-06-15 00:00:00
abstract::Complex I (CI) is the largest component of the mitochondrial respiratory chain (MRC) and it is made up of 7 mitochondrial DNA (mtDNA)-encoded and at least 38 nuclear DNA-encoded subunits. Isolated CI deficiency is the most common single enzyme deficiency in the heterogeneous group of MRC disorders and it is a relative...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2019.02.010
更新日期:2019-04-15 00:00:00
abstract::Previously, we demonstrated that neuronal nitric oxide synthase (nNOS) is activated and promotes muscle atrophy in skeletal muscle during tail suspension, a model of unloading and denervation. Here, we examined patients with amyotrophic lateral sclerosis (ALS) and mutant (H46R) SOD1 transgenic (Tg) mice model using im...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2010.03.022
更新日期:2010-07-15 00:00:00
abstract:BACKGROUND:Glucosamine (UDP-N-acetyl)-2-epimerase/N-acetylmannosamine kinase (GNE) myopathy, also called distal myopathy with rimmed vacuoles (DMRV) or hereditary inclusion body myopathy (HIBM), is a rare, progressive autosomal recessive disorder caused by mutations in the GNE gene. Here, we examined the relationship b...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2012.03.016
更新日期:2012-07-15 00:00:00
abstract::Peripheral nerve injury results in a series of events culminating in degradation of the axonal cytoskeleton (Wallerian degeneration). In the time period between axotomy and cytoskeletal degradation (24-48 h in rodents), there is calcium entry and activation of calpains within the axon. The precise timing of these even...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(02)00013-8
更新日期:2002-04-15 00:00:00
abstract::Despite the involvement of cerebellar ataxia in a large variety of conditions and its frequent association with other neurological symptoms, the quantification of the specific core of the cerebellar syndrome is possible and useful in Neurology. Recent studies have shown that cerebellar ataxia might be sensitive to var...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(96)00231-6
更新日期:1997-02-12 00:00:00
abstract:INTRODUCTION:Intracerebral hemorrhage (ICH) associated with pregnancy commonly occurs in the postpartum period in the setting of preeclampsia/eclampsia. We describe the clinical course of two patients with ICH due to postpartum cerebral vasculopathy in the absence of toxemia. METHODS:We reviewed two cases with ICH and...
journal_title:Journal of the neurological sciences
pub_type: 临床试验,杂志文章
doi:10.1016/s0022-510x(02)00245-9
更新日期:2002-12-15 00:00:00
abstract::We have used light microscopic histomorphometry to quantify the developmental histopathological changes induced by muscular dystrophy in the soleus and extensor digitorum longus (EDL) muscles of the mdx mouse. We find that this X-linked disease exhibits early fibre necrosis with foci of invasive cells, clustering of a...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(87)90219-x
更新日期:1987-08-01 00:00:00
abstract:BACKGROUND:Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary disorder caused by NOTCH3 mutations, usually localized to exons 3 and 4, and characterized by recurrent subcortical infarctions, dementia and leukoencephalopathy. So far, there has been only l...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2006.02.011
更新日期:2006-07-15 00:00:00
abstract::Although fatigue is a frequent complaint of patients with multiple sclerosis (MS), little is known about the origins of multiple-sclerosis-associated fatigue. Our primary focus was to study if the extent of cerebral abnormalities, as shown on magnetic resonance imaging (MRI), had any relation with the frequency and in...
journal_title:Journal of the neurological sciences
pub_type: 临床试验,杂志文章
doi:10.1016/s0022-510x(98)00251-2
更新日期:1998-10-08 00:00:00
abstract::The pattern of mental dysfunction in multiple sclerosis (MS) is characteristic of the so-called subcortical dementia. Cognitive dysfunction results predominantly by the disruption of communication among cortical and subcortical areas, as a consequence of the white matter damage. As expected, studies with conventional ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/s0022-510x(99)00282-8
更新日期:2000-01-15 00:00:00
abstract::The phase of rapid reduction of isometric strength in single muscle groups was identified and its slope was calculated in patients studied with the TQNE method. This parameter was studied in the extremities and in respiration in 4 ALS patients and 4 PMA (progressive spinal muscle atrophy) patients. The slopes of the s...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(96)00121-9
更新日期:1996-08-01 00:00:00
abstract::Autoantibodies against astrocyte water channel aquaporin-4 (AQP4) are highly specific for neuromyelitis optica (NMO). However, the molecular mechanism of NMO still remains unclear. The purpose of this study was to identify the possible humoral mechanisms responsible for the occurrence of astrocytic damage. Human prima...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2013.05.035
更新日期:2013-08-15 00:00:00
abstract::This study explores the severity of depression and anxiety experienced by patients suffering from motor neurone disease and seeks to link this to illness progression, recent life events and personality factors. The 18 patients who participated in the study were assessed for the presence of psychiatric symptoms using t...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(98)00203-2
更新日期:1998-10-01 00:00:00
abstract::The presence of postural disturbance in patients with Parkinson's disease (PD) was assessed by the displacement of the centre of foot pressure (CFP) and by changes in the amplitude of the soleus H-reflex when patients maintained an upright standing posture, followed by a forward-leaning posture. Thirteen patients and ...
journal_title:Journal of the neurological sciences
pub_type: 临床试验,杂志文章
doi:10.1016/s0022-510x(97)00175-5
更新日期:1997-12-09 00:00:00
abstract::We examined 19 muscle biopsies from 14 patients with autosomal recessive distal muscular dystrophy (DisMD) histochemically and immunohistochemically to characterize the histologic features of this disease and to determine whether dystrophin and dystrophin-associated proteins (DAPs) are normally present in the muscles ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(94)90096-5
更新日期:1994-10-01 00:00:00
abstract::Using EMG equipment with time locked recording and standard averaging facilities we were able to record a previously poorly defined skin potential. The potential, termed by us the peripheral autonomic surface potential (PASP), was recorded from the palmar surface of the hand and the plantar surface of the foot in 30 n...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(85)90120-0
更新日期:1985-02-01 00:00:00
abstract::To determine the prevalence and subtypes of dementia in southern Taiwan, a two-phase study consisting of a phase I screening survey using the Mini-Mental Status Examination (MMSE) and a phase II diagnostic examination using the CERAD neuropsychological battery and the neurobehavioral examination was conducted. Accordi...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(98)00225-1
更新日期:1998-09-18 00:00:00
abstract:OBJECTIVE:To determine the influence of different factors on test-retest reliability of frequently used transcranial magnetic stimulation (TMS) parameters while controlling for potential confounders in healthy subjects. METHODS:TMS was applied in 93 healthy volunteers (61% male) twice (mean retest interval of 34.0 ± 2...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2016.01.039
更新日期:2016-03-15 00:00:00
abstract::The prevalence and pattern of cognitive impairment in systemic lupus erythematosus (SLE) patients with (NPSLE) and without (nSLE) overt neuropsychiatric manifestations were investigated. Fifty-two nSLE patients, 23 NPSLE patients and 27 healthy controls were evaluated with a battery of standardized neuropsychological ...
journal_title:Journal of the neurological sciences
pub_type: 临床试验,杂志文章
doi:10.1016/s0022-510x(00)00492-5
更新日期:2001-02-15 00:00:00
abstract::Systemic inflammatory response syndrome (SIRS) is a medical condition in which the all-organ microcirculation is affected including nervous system. We describe neurological findings in 64 patients with SIRS at Hospital das Clínicas of Sao Paulo University School of Medicine; 45.3% were male and 54.7% female; their age...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2006.08.015
更新日期:2006-12-01 00:00:00
abstract::Further observations on a family with facioscapulohumeral (FSH) muscular dystrophy due to mitochondrial myopathy, and on a case with lipid storage myopathy are reported. One member of the family with FSH muscular dystrophy died due to a viral pneumonia, during which she developed gross hyperlacticacidaemia and acidosi...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(78)90003-5
更新日期:1978-02-01 00:00:00
abstract::A study was made of Wistar rat soleus muscle following intraperitoneal administration of denervated muscle extract over 1 and 2 days. Light microscopy revealed the appearance on fiber surfaces of basophilic satellite structures whose histochemical behaviour differed from that of the parent fiber. Small fibers showing ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(94)00212-7
更新日期:1995-01-01 00:00:00
abstract::A method for preparative separation of cerebrospinal fluid specific proteins by affinity chromatography and isoelectric focusing is described. It has been tested on CSF from a group of human patients suffering from TIA. The advantage of the method is the production of a protein pattern easy to survey by eye. The sensi...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(80)90132-x
更新日期:1980-01-01 00:00:00