Abstract:
:The protein compositions of subcellular fractions of muscle obtained from 17 Duchenne dystrophy patients, 15 disease controls (10 different primary myopathies, 5 spinal muscular atrophy patients), and 10 normals were examined by polyacrylamide gel electrophoresis. The gels were stained with Coomassie Brilliant Blue and with "Stains-all," which stains calcium-binding proteins, sialic acid-rich glycoproteins, and phosphoproteins. In muscle membrane fractions of Duchenne dystrophy patients there was a marked reduction in the concentrations of calsequestrin and a 39 kDa protein that stained blue with "Stains-all." There were changes in the proteins of all subcellular fractions of Duchenne's patients; some of these changes appear to be specific for Duchenne dystrophy (DD). There was no apparent correlation between the protein changes observed on acrylamide gels and the age of the patients, the duration of the disease, the degree of disability, or activity of creatine kinase. A decreased level of calsequestrin in DD sarcoplasmic reticulum may contribute to an increased level of free calcium seen in muscle from these patients.
journal_name
Muscle Nervejournal_title
Muscle & nerveauthors
Niebroj-Dobosz I,Kornguth S,Schutta H,Siegel FL,Hausmanowa-Petrusewicz Idoi
10.1002/mus.880120404subject
Has Abstractpub_date
1989-04-01 00:00:00pages
273-80issue
4eissn
0148-639Xissn
1097-4598journal_volume
12pub_type
杂志文章相关文献
MUSCLE & NERVE文献大全abstract:INTRODUCTION:Merkel cell carcinoma is a rare cutaneous, aggressive tumor. Although it shares many neuroendocrine features with small cell lung carcinoma, it has only occasionally been reported with paraneoplastic neurological syndromes. METHODS:A healthy 67-year-old man developed acute ataxia, vertigo, and nausea. Sub...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.25530
更新日期:2017-11-01 00:00:00
abstract::The values obtained from two different macro EMG methods were compared, and found to be different. Normally, macro electromyography (EMG) is performed with a modified single fiber (SF) needle using the SF potential as a trigger; a new method recommends a modified concentric needle and triggers on the concentric EMG si...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880150211
更新日期:1992-02-01 00:00:00
abstract::Calpains are Ca2+ -dependent cytosolic cysteine proteases that participate in the pathology of Duchenne muscular dystrophy (DMD). Utrophin is a functional homolog of dystrophin that partially compensates for dystrophin deficiency in myofibers of mdx mice. In this study, we investigated the susceptibility of utrophin t...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20549
更新日期:2006-06-01 00:00:00
abstract::Excitability properties were recorded from 14 volunteers following stimulation of the recurrent motor branch of the median nerve in the palm. Distal stimulation resulted in significantly lower strength-duration time constant and lower threshold during prolonged hyperpolarization than did wrist stimulation in the same ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.1205
更新日期:2001-12-01 00:00:00
abstract::Acetylcholinesterase (AChE) and butyrylcholinesterase (BuChE) were studied in the diaphragm of early postnatal rats, using radiometric determination of enzyme activities, velocity sedimentation for separation of molecular forms, and electron microscopic cytochemistry to localize enzymes. AChE activity did not change s...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880040607
更新日期:1981-11-01 00:00:00
abstract:INTRODUCTION:Bifacial weakness with paresthesias is a rare subtype of Guillain-Barré syndrome (GBS), characterized by facial diplegia in the absence of any other cranial neuropathies, limb weakness, or ataxia. Frequently, patients also complain of distal limb paresthesias before or at the time they develop facial weakn...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.25028
更新日期:2016-05-01 00:00:00
abstract::Peripheral neuropathies are reported to arise as a result of the systemic inflammatory response produced by a full-thickness cutaneous burn injury. This study was designed to characterize the magnitude and time course of functional and morphological changes in peripheral axons that arise after a full-thickness dermal ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20306
更新日期:2005-05-01 00:00:00
abstract:INTRODUCTION:In Duchenne muscular dystrophy (DMD), fat replacement of muscle may be a useful endpoint in trials of therapy, although progression in different muscle groups is uneven. In this study we assessed the progression of fat replacement with T(1) -weighted imaging over 2 9-month periods. METHODS:Eight ambulant,...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.23879
更新日期:2013-10-01 00:00:00
abstract:INTRODUCTION:The involvement of small myelinated fibers in the early stages of diabetic neuropathy (DN) has been suggested, but few objective methods can identify minimal neuropathy. The purpose of this study was to determine whether there are differences in contact heat evoked potential (CHEP) parameters between healt...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.22192
更新日期:2011-12-01 00:00:00
abstract:INTRODUCTION:Electrical impedance myography (EIM) is an emerging non-invasive, highly reproducible electrophysiological technique that objectively characterizes muscle structure and composition by measuring bioimpedance. We assessed the ability of EIM ability to discriminate 2 forms of congenital muscular dystrophy (CM...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.24770
更新日期:2016-03-01 00:00:00
abstract::Patients (n = 41) with isolated weakness of the eyelids or extraocular muscles, who had been referred for single fiber electromyography (SFEMG), were followed up after 4 to 24 months, At follow-up the patients were classified as "definite ocular myasthenia gravis" (MG), "definite other diagnosis," or "no definite diag...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880150322
更新日期:1992-03-01 00:00:00
abstract:INTRODUCTION:We sought to determine whether electrical impedance myography (EIM) could serve as a diagnostic procedure for evaluation of radiculopathy. METHODS:Twenty-seven patients with clinically and radiologically diagnosed cervical or lumbosacral radiculopathy who met a "gold standard" definition underwent EIM and...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.23833
更新日期:2013-11-01 00:00:00
abstract:BACKGROUND:Limited data exist regarding myopathies with early or prominent dysphagia. METHODS:A retrospective study was performed (January 2003 to August 2019) to identify myopathy patients in whom dysphagia was the initial symptom or was disproportionately severe compared with limb weakness. RESULTS:Thirty-two patie...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.26996
更新日期:2020-09-01 00:00:00
abstract::Clinical testing for neuromuscular dysfunction is supported by an extensive amount of excellent basic information about normal and abnormal subcellular physiology and ultrastructure. This information provides an essential frame of reference for describing the rationale of single-fiber electromyography (SFEMG). SFEMG i...
journal_title:Muscle & nerve
pub_type: 杂志文章,评审
doi:10.1002/mus.880120802
更新日期:1989-08-01 00:00:00
abstract::Present studies evaluated the functions of thick and thin nerve fibers in carpal tunnel syndrome (CTS) to determine whether data of thin nerve fibers can add further information to the diagnosis of CTS. Measurements were performed in 22 patients and 16 age-matched control subjects. Motor and sensory nerve conduction, ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880180210
更新日期:1995-02-01 00:00:00
abstract::Epstein-Barr virus infection can affect both the central and peripheral nervous system. In some patients this occurs concurrently. Two patients are presented with encephalopathy and acute quadriparesis with diminished reflexes. Positive serology for Epstein-Barr virus was found in both patients. Both patients had a ce...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/(SICI)1097-4598(199608)19:8<1037::AID-MUS1
更新日期:1996-08-01 00:00:00
abstract::The Moberg Pick-Up Test is a standardized test for assessing hand dexterity. Although reduction of sensation in the hand occurs with aging, the effect of age on a subject's performance of the Moberg Pick-Up Test has not been examined. The primary goal of this study was to examine the impact of aging and, secondarily, ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20750
更新日期:2007-06-01 00:00:00
abstract:INTRODUCTION:Widespread musculoskeletal pain is a well-known symptom of myotonic dystrophy type 2 (DM2), but so far it has been addressed in only a few studies. METHODS:A postal survey for all traceable DM2 patients (n = 132) was conducted. A specific questionnaire, and severity and interference subscales of the Brief...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.22249
更新日期:2012-01-01 00:00:00
abstract:INTRODUCTION:Nebulin is a giant actin-binding protein in the thin filament of the skeletal muscle sarcomere. Studies of nebulin interactions are limited by the size, complexity, and poor solubility of the protein. We divided the nebulin super-repeat region into a super-repeat panel, and studied nebulin/actin interactio...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.26350
更新日期:2019-01-01 00:00:00
abstract:INTRODUCTION:Muscle fatigue could have a greater impact on position sense when antagonists of the movement are fatigued. Hence, this study aimed to compare the effects of antagonist and agonist exercise-induced muscle fatigue on knee joint position sense. METHODS:This within-subjects repeated-measures study included 4...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.22018
更新日期:2011-08-01 00:00:00
abstract::We describe a patient with transthyretin amyloidosis who presented with multifocal mononeuropathies with features of demyelination on nerve conduction studies, a constellation of findings not previously described in amyloid polyneuropathy. Genetic testing revealed a valine122isoleucine mutation in the coding region of...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.10614
更新日期:2004-02-01 00:00:00
abstract::Median neuropathies proximal to the wrist are uncommon and usually result from penetrating injuries, fracture dislocation of the distal humerus, or compression by fibrous bands. A 66-year-old man suffered a comminuted fracture of the proximal humerus after a fall. Electrodiagnostic studies revealed a severe proximal m...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/(sici)1097-4598(200003)23:3<426::aid-mus15
更新日期:2000-03-01 00:00:00
abstract::During postnatal development of extensor digitorum longus (EDL) muscle, sodium withdrawal contractures were observed during the first 6 days after birth, and not after this time. In regenerating EDL muscles, zero-Na contractures were demonstrated: (1) 7 days after bupivacaine injection, but not 14 or 90 days after thi...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/(sici)1097-4598(199811)21:11<1530::aid-mus
更新日期:1998-11-01 00:00:00
abstract:INTRODUCTION:In this study we present a reproducible technique to assess motor recovery after nerve injury via neuromuscular junction (NMJ) immunostaining and electrodiagnostic testing. METHODS:Wild-type mice underwent sciatic nerve transection with repair. Hindlimb muscles were collected for microscopy up to 30 weeks...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.26654
更新日期:2019-11-01 00:00:00
abstract::Mutations of the valosin-containing protein gene (VCP) are responsible for autosomal-dominant hereditary inclusion-body myopathy associated with frontotemporal dementia and Paget's disease of bone. We identified the p.R155C missense mutation in the VCP gene segregating in an Italian family with three affected siblings...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20890
更新日期:2008-01-01 00:00:00
abstract::Electron microscopic studies of long-term denervated rat muscles have identified very small, immature myofibers that are believed to arise from detached satellite cells that have fused to form new fibers within the interstitial space. At present, it is unknown whether and to what extent equivalent fibers exist in dene...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20902
更新日期:2008-01-01 00:00:00
abstract:INTRODUCTION:Sarcopenia likely comprises muscle fiber denervation and re-innervation, resulting in clustering of muscle fibers of the same type (classified by myosin heavy chain isoform composition). Development of methodology to quantitatively evaluate clustering of muscle fibers according to fiber type is necessary. ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.24641
更新日期:2015-07-01 00:00:00
abstract::Focal entrapment of the ulnar nerve occurs most frequently in the region of the elbow, at the ulnar groove or beneath the humeroulnar aponeurosis. Surgical treatment commonly involves transposition of the nerve anterior to the medial epicondyle, in the antecubital fossa. Symptoms may recur after surgery, and, to asses...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20103
更新日期:2004-10-01 00:00:00
abstract::We developed electrophysiologic methods for testing the three major forelimb nerves as a tool to evaluate motor and sensory recovery in rats. Median, ulnar, or radial nerves were transected and repaired in Sprague-Dawley rats. Compound muscle action potentials (CMAPs) and somatosensory evoked potentials (SSEPs) were r...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20971
更新日期:2008-10-01 00:00:00
abstract::Comparison was made between simultaneous recordings of the electromyogram (EMG) and acoustic myogram (AMG) signals and the torque produced by the lumbar erectores spinae muscles of three healthy subjects while they performed isometric contractions. Repeatability of each signal was studied. The AMG had a relationship t...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880110409
更新日期:1988-04-01 00:00:00