Abstract:
:Recently described techniques for separating myosin isoenzymes have been adapted for analysis of myosins from diseased and developing human skeletal muscle. The method is highly suitable for analysis of human myosins because only 2 - 3 mg of muscle are required for routine analyses. Human embryonic/foetal myosins are electrophoretically distinct from mature skeletal myosins, and are not normally detected beyond the first month of post-natal life, except in premature infants. They have a high alkaline calcium-activated ATPase activity. This would account for the histochemical classification of foetal fibres as "Type II", although physiological differences between adult fast-twitch muscle and foetal muscle are well recognized. Foetal myosins are also synthesized in human skeletal muscle under certain pathological circumstances. Their presence in Duchenne dystrophy probably reflects the associated marked muscle regeneration, with immaturity of some muscle cells. The large amounts of foetal myosin present in many cases of infantile spinal muscular atrophy is evidence that innervation is necessary for the normal cessation of foetal myosin synthesis.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Fitzsimons RB,Hoh JFdoi
10.1016/0022-510x(81)90018-6subject
Has Abstractpub_date
1981-11-01 00:00:00pages
367-84issue
2-3eissn
0022-510Xissn
1878-5883pii
0022-510X(81)90018-6journal_volume
52pub_type
杂志文章abstract::We report on the case of a 70-year-old man with primary macroglobulinemia who showed cranial polyneuropathy and extensive radiculoneuropathy. His serum contained an IgM lambda monoclonal antibody which reacted with both a high molecular weight protein in grey matter and purified myelin basic protein (MBP) on immunoblo...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(96)05305-1
更新日期:1997-05-01 00:00:00
abstract:BACKGROUND:Although a number of studies reported different interictal findings between migraine with aura (MA) and migraine without aura (MO), the pathophysiology of the visual aura in migraine remains unclear. OBJECTIVE:To investigate the visual processing in patients who experience MA between attacks using steady-st...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2008.04.004
更新日期:2008-08-15 00:00:00
abstract::Cutaneous reflex (CR) was measured in 15 patients with acute lacunar stroke and in 15 age- and sex-matched normal subjects. EMG activities were recorded from the first dorsal interosseous muscle with surface electrodes, rectified and averaged. Intensity of the stimulation on the index finger was four times the sensory...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(98)00142-7
更新日期:1998-07-15 00:00:00
abstract::The amyloid precursor protein (APP) in brain is processed either by an amyloidogenic pathway by beta-secretase and gamma-secretase to yield Abeta (beta-amyloid 4 kDa) peptide or by alpha-secretase within the beta-amyloid domain to yield non-amyloidogenic products. We have studied blood platelet levels of a 22-kDa frag...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2005.09.002
更新日期:2006-01-15 00:00:00
abstract::Guillain-Barré syndrome (GBS) is considered a monophasic disorder yet recurrences occur in up to 6% of patients. We retrospectively studied an Italian-Japanese population of 236 GBS and 73 Miller Fisher syndrome (MFS) patients and searched for factors which may be associated with recurrence. A recurrent patient was de...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2016.02.053
更新日期:2016-06-15 00:00:00
abstract::Data of 2399 brain weights and ages from populations before 1880, 1885 to 1900 and 1966 to 1976 were obtained from German anatomical and pathological institutes, analyzed with non-linear and multiple linear regression analyses and the results compared. The influence of the absolute age (sample period) on brain weights...
journal_title:Journal of the neurological sciences
pub_type: 历史文章,杂志文章
doi:10.1016/0022-510x(79)90202-8
更新日期:1979-02-01 00:00:00
abstract::The ability of galantamine (Reminyl) to inhibit the aggregation and toxicity of the beta-amyloid peptide (Abeta) was investigated. Galantamine showed concentration-dependent inhibition of aggregation of both Abeta 1-40 and Abeta 1-42, as determined by an ELISA method. Electron microscope studies of Abeta 1-40 incubate...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2009.01.024
更新日期:2009-05-15 00:00:00
abstract::Selenoprotein N-related myopathy (SEPN1-RM) is an early-onset muscle disorder that can manifest clinically as congenital muscular dystrophy with spinal rigidity and can result in specific pathological entities such as multiminicore disease, desmin-related myopathy with Mallory body-like inclusions, and congenital fibe...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2010.09.011
更新日期:2011-01-15 00:00:00
abstract::To investigate the underlying basis for the selective modulation of the permeability of the blood-brain barrier by small doses of bradykinin, we first established cell lines of rat brain microvascular endothelial cells (BMECs) and astrocytes by primary cultures from neonatal rats. BMECs, astrocytes and C6 glioma cells...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2007.02.031
更新日期:2007-07-15 00:00:00
abstract::Neocortical lesions (NLs) are an important component of multiple sclerosis (MS) pathology and may account for part of the physical and cognitive disability. Visualizing NLs of patients with MS using magnetic resonance imaging (MRI) poses several significant challenges. We optimized the inversion time (TI) of T(1)-base...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2009.03.021
更新日期:2009-07-15 00:00:00
abstract::Frontal gaits (FG) and parkinsonian gaits (PG) are common neurological gait abnormalities in older adults. It may be difficult to distinguish these gaits as they share common clinical characteristics such as unsteadiness, slowing, and shuffling. Of 488 community-residing subjects in an aging study, 11 were diagnosed w...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2006.05.035
更新日期:2006-10-25 00:00:00
abstract::Abnormal function of apolipoprotein E (apoE) has been implicated in the incidence of some neurological disorders including dementia. Our recent experiments have shown that apoE deficiency alters the dynamics of alpha tocopherol (vitamin E) handling by brain. In the current investigation, we examined the uptake and ret...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2009.02.350
更新日期:2009-08-15 00:00:00
abstract::Superficial siderosis (SS) of the central nervous system is a rare disorder caused by chronic or recurrent hemorrhages into the subarachnoid space with hemosiderin and ferritin deposition, which leads to neuronal damage. The source of bleeding remains unknown in 50% of cases. Recently, attention has been focused on fl...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2011.07.034
更新日期:2012-01-15 00:00:00
abstract:OBJECTIVE:Stiff person syndrome (SPS) is usually characterized by truncal muscle rigidity and episodic painful spasms, but it sometimes appears with ocular symptoms called "stiff eyes". We recorded saccade movements in an SPS patient manifesting with "stiff eyes" conditions with slow saccade velocity and evaluated the ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2019.116506
更新日期:2020-01-15 00:00:00
abstract::Sensory ataxia in inorganic germanium intoxication is rare. A 63-year-old housewife had taken inorganic germanium preparations at a dosage of 36 mg a day for about 6 years (total dose about 80 g). She subsequently developed difficulty in writing and gait disturbance with peripheral neuropathy and renal involvement. Ge...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(95)00032-w
更新日期:1995-06-01 00:00:00
abstract:BACKGROUND:Motor impairment in Parkinson's disease (PD) includes slowness (bradykinesia), decreased amplitude (hypokinesia), impaired rhythm and a progressive reduction in speed and amplitude during movement repetition (sequence effect). In the present study we aimed to analyse bradykinesia features in newly-diagnosed ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2016.08.028
更新日期:2016-10-15 00:00:00
abstract::Functional and metabolic recovery of a female cat is described which survived for 1 year following 1 hour global cerebro-circulatory arrest at normothermia. Ischemia was produced by intrathoracal occlusion of the innominate, the left subclavian and both mammary arteries. Following ischemia the animal was kept under in...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(87)90130-4
更新日期:1987-02-01 00:00:00
abstract::Weanling male Wistar rats fed a diet containing 0.5-2% cuprizone developed intramyelinic edema of the cerebellar white matter, hilum of the dentate nucleus and superior cerebellar peduncle. Oligodendrocytes in these regions showed hyperchromatic nuclei and abnormally dense cytoplasm, with an increase in the number of ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(88)90127-x
更新日期:1988-04-01 00:00:00
abstract::High resolution positron emission tomography (PET) with the newly developed HRRT scanner (Siemens/CTI) permits the reliable quantification of 18-Fluorodeoxyglucose (FDG) uptake as a marker of neuronal activity in small subcortical nuclei which are involved in the pathophysiology of Parkinson's disease (PD). We investi...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2008.08.029
更新日期:2009-01-15 00:00:00
abstract::We herein report a 12-year-old girl with a basal ganglia germinoma who presented with right-sided hemiparesis after a minor head trauma. Magnetic resonance (MR) imaging revealed a minimally enhanced lesion involving the left putamen, thalamus, and corona radiata. The lesion showed low-signal intensity on T1-, and high...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2008.02.006
更新日期:2008-07-15 00:00:00
abstract::Two transgenic strains of mutant mice lacking hypoxanthine-guanidine phosphoribosyltransferase (HPRT) activity were examined behaviorally and neurochemically for phenotypic similarity to the human Lesch-Nyhan syndrome. In this syndrome, male children markedly deficient in the enzyme HPRT develop self-mutilation and se...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(88)90099-8
更新日期:1988-09-01 00:00:00
abstract::In Creutzfeldt-Jakob disease (CJD), there are prominent ultrastructural alterations of the plasma membrane, which contains many glycolipids and glycoproteins. Glycosidases can degrade glycolipids and glycoproteins. Gangliosides, a subset of glycolipids, are decreased in amount at the terminal stages of CJD, and CJD in...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(88)90178-5
更新日期:1988-03-01 00:00:00
abstract::A number of diseases exhibit neurodegeneration with/without additional symptoms such as immunodeficiency, increased cancer risk, and microcephalus. Ataxia telangiectasia and Nijmegen breakage syndrome, for example, develop as a result of mutations in genes involved in the DNA damage response. However, such diseases ca...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2014.02.033
更新日期:2014-05-15 00:00:00
abstract::The study is designed to assess the oxidative stress intensity in erythrocytes obtained from patients in different clinical phenotypes of neuroinflammation, defined as clinically isolated syndrome (CIS) and relapsing-remitting multiple sclerosis (RRMS). Advanced oxidation protein products (AOPP), malondialdehyde (MDA)...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2013.11.006
更新日期:2014-02-15 00:00:00
abstract::Medial temporal lobe epilepsy is commonly associated with hippocampal atrophy on MRI and hippocampal sclerosis on histopathological examination of surgically-resected specimens. Likewise, it is well-established that prolonged seizures and status epilepticus can lead to hippocampal edema as noted on MRI. In this paper,...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2013.08.016
更新日期:2013-11-15 00:00:00
abstract::Administration of retinoic acid (RA) prevented the development of experimental allergic encephalomyelitis (EAE) in Lewis rats. RA suspended in corn oil was given for 3 days before the expected onset of the disease to rats immunized with myelin and to controls. The drug suppressed the neurological symptoms as well as t...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(87)90220-6
更新日期:1987-08-01 00:00:00
abstract:OBJECTIVE:To explore the role of cardiovascular risk factors and the different NOTCH-3 mutations to explain the variability observed in the clinical presentation of CADASIL. METHODS:This was a retrospective cohort study of 331 individuals, 90 were carriers of four mutations in the NOTCH3 gene. These four mutations are...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2020.117178
更新日期:2020-12-15 00:00:00
abstract::The levels of the neuroprotective excitatory amino acid receptor antagonist kynurenic acid (KYNA) have been previously shown to be reduced in several regions of the brain of Huntington's disease (HD) patients. Thus, KYNA has been speculatively linked to the pathogenesis of HD. We have examined KYNA levels and the acti...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(94)00280-2
更新日期:1995-05-01 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::Hemifacial spasm is characterised by unilateral contractions of the facial muscles. Though considered to be benign by many people, it can lead to functional blindness and a poor quality of life due to social embarrassment for the suffering individual. Botulinum toxin therapy is an excellent noninvasive tool to treat t...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/j.jns.2015.06.032
更新日期:2015-09-15 00:00:00