Nerve conduction abnormalities in aging mice deficient for myelin-associated glycoprotein.

Abstract:

:Ultrastructural, biochemical, and electrophysiological analyses were done on 12-14-month-old mice deficient for myelin-associated glycoprotein (MAG) to further characterize the neuropathy that develops as they age. Electron microscopy demonstrated normal myelin compaction and axonal degeneration in a large number of myelinated nerve fibers. Western blots showed that the proteins of compact myelin, P0 glycoprotein, and myelin basic protein were not significantly altered in the mutants; however, the Schwann cell protein, 2',3'-cyclic nucleotide 3'-phosphodiesterase, was reduced to less than half the control level. Also, both total and phosphorylated high-molecular-weight neurofilament proteins (TNFH and PNFH, respectively) were significantly decreased, as was the PNFH:TNFH ratio. Electrophysiological evaluation revealed a mild, but statistically significant, reduction of conduction velocity and a nonsignificant mild decrease in compound muscle action potential amplitudes. This constellation of findings in aging MAG-null mice is consistent with an axonopathy that resembles axonal Charcot-Marie-Tooth (CMT2) disease in many respects. Thus, mutation of a myelin-associated gene expressed by Schwann cells can induce axonal degeneration and cause a neuropathy with minimal signs of demyelination.

journal_name

Muscle Nerve

journal_title

Muscle & nerve

authors

Weiss MD,Luciano CA,Quarles RH

doi

10.1002/mus.1159

subject

Has Abstract

pub_date

2001-10-01 00:00:00

pages

1380-7

issue

10

eissn

0148-639X

issn

1097-4598

pii

10.1002/mus.1159

journal_volume

24

pub_type

杂志文章
  • The utility of various sensory nerve conduction responses in assessing brachial plexopathies.

    abstract::To determine which sensory nerve conduction studies (S-NCS) are helpful in detecting supraclavicular axon loss brachial plexopathies, we selected 53 cases (of 417 reviewed) in whom complicating factors were absent and which, by needle electrode examination findings, involved only a single "truncal" element (upper, mid...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880180813

    authors: Ferrante MA,Wilbourn AJ

    更新日期:1995-08-01 00:00:00

  • Cytoplasmic body myopathy masquerading as motor neuron disease.

    abstract::Cytoplasmic body myopathy (CBM) is characterized by proteinaceous inclusion bodies in muscle tissue. A 43-year-old woman presented with rapidly progressive weakness and dysphagia. Electromyography (EMG) elsewhere demonstrated lower-limb chronic partial denervation. Muscle biopsy showed fiber size variation without dia...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.20115

    authors: Krishnan AV,Pamphlett R,Burke D,Wills EJ,Kiernan MC

    更新日期:2004-11-01 00:00:00

  • Inhibition of dynamic thigh muscle contraction by electrical stimulation of the posterior cruciate ligament in humans.

    abstract::We investigated the influence of electrical stimulation of the posterior cruciate ligament (PCL) on the motoneuron pool of the thigh muscle during voluntary static and dynamic muscle contraction. The study group comprised nine young men with no history of injury to the knee joints. Multistranded Teflon-insulated stain...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.1172

    authors: Fischer-Rasmussen T,Krogsgaard M,Jensen DB,Dyhre-Poulsen P

    更新日期:2001-11-01 00:00:00

  • Health-related quality-of-life and treatment targets in myasthenia gravis.

    abstract:INTRODUCTION:The aim of this study was to determine factors affecting health-related quality of life (HRQOL) and to propose appropriate treatment targets for patients with myasthenia gravis (MG). METHODS:We evaluated 640 consecutive patients with MG seen at 11 neurological centers. Two-year follow-up data were obtaine...

    journal_title:Muscle & nerve

    pub_type: 杂志文章,多中心研究

    doi:10.1002/mus.24213

    authors: Utsugisawa K,Suzuki S,Nagane Y,Masuda M,Murai H,Imai T,Tsuda E,Konno S,Nakane S,Suzuki Y,Fujihara K,Suzuki N

    更新日期:2014-10-01 00:00:00

  • Clinical and electrophysiological features of the 2007 Guillain-Barré syndrome epidemic in northeast China.

    abstract::Guillain-Barré syndrome (GBS) generally presents sporadically. Epidemics of GBS are unusual. We reviewed the medical records of 26 patients hospitalized for GBS during the 2007 GBS epidemic in northeast China. The objective was to determine whether there were clinical and electrophysiological characteristics. All pati...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.21701

    authors: Ye Y,Zhu D,Wang K,Wu J,Feng J,Ma D,Xing Y,Jiang X

    更新日期:2010-09-01 00:00:00

  • Relationship between electrical and vibratory output of muscle during voluntary contraction and fatigue.

    abstract::Measurements were done on the biceps muscles of 6 healthy volunteers to record simultaneously the surface electromyogram (EMG) and vibromyogram (VMG) by means of a piezoelectric device (accelerometer). The VMG is generated by mechanical waves due to the contraction mechanism and often measured as sound. The frequency ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880140810

    authors: Zwarts MJ,Keidel M

    更新日期:1991-08-01 00:00:00

  • Developing normalized strength scores for neuromuscular research.

    abstract:INTRODUCTION:The Accurate Test of Limb Isometric Strength (ATLIS) device can reliably measure the strength of 12 muscle groups using a fixed load cell. The purpose of this study was to analyze ATLIS data from healthy adults to calculate an individual's predicted strength scores. METHODS:ATLIS data were collected from ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.23516

    authors: Andres PL,English R,Mendoza M,Florence J,Malkus E,Schierbecker J,Siener C,Malspeis S,Schoenfeld DA,Munsat TL,Cudkowicz ME

    更新日期:2013-02-01 00:00:00

  • Prospective comparison of acute motor axonal neuropathy and acute inflammatory demyelinating polyradiculoneuropathy in 140 children with Guillain-Barré syndrome in India.

    abstract:INTRODUCTION:There have been few reports on subtypes of Guillain-Barré syndrome (GBS) in children. We compared clinical and laboratory findings of acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). METHODS:One hundred forty children with GBS were included. Based on...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25992

    authors: Kalita J,Kumar M,Misra UK

    更新日期:2018-05-01 00:00:00

  • Miconazole enhances nerve regeneration and functional recovery after sciatic nerve crush injury.

    abstract:INTRODUCTION:Improving axonal outgrowth and remyelination is crucial for peripheral nerve regeneration. Miconazole appears to enhance remyelination in the central nervous system. In this study we assess the effect of miconazole on axonal regeneration using a sciatic nerve crush injury model in rats. METHODS:Fifty Spra...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.26033

    authors: Lin T,Qiu S,Yan L,Zhu S,Zheng C,Zhu Q,Liu X

    更新日期:2018-05-01 00:00:00

  • Relative contributions of neural mechanisms versus muscle mechanics in promoting finger extension deficits following stroke.

    abstract::The origins of impaired finger and hand function were examined in 10 stroke survivors with chronic spastic hemiparesis, with the intent of assessing whether mechanical restraint or altered neurophysiological control mechanisms are responsible for the well-known impairment of finger extension. Simultaneous extension of...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.10443

    authors: Kamper DG,Harvey RL,Suresh S,Rymer WZ

    更新日期:2003-09-01 00:00:00

  • Demyelinating neuropathy with anti-CRMP5 antibodies predating diagnosis of breast carcinoma: favorable outcome after cancer therapy.

    abstract:INTRODUCTION:Breast cancer is exceptionally associated with anti-collapsin response mediator protein 5 (anti-CRMP5) antibody or demyelinating neuropathy. This paraneoplastic antibody is itself not associated with demyelinating neuropathy. METHODS:Herein we describe a patient with a predominantly sensory ataxic demyeli...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.22036

    authors: Samarasekera S,Rajabally YA

    更新日期:2011-05-01 00:00:00

  • Multifocal motor neuropathy with conduction block misdiagnosed as multiple entrapment neuropathies.

    abstract::We describe a 58-year-old male with a few years history of multifocal weakness in the upper limbs with minimal to absent sensory complaints. He was diagnosed as having multiple compressive neuropathies, which required repeated decompressive surgeries. Electrodiagnostic studies prior to diagnosis were limited to a few ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/(sici)1097-4598(199806)21:6<813::aid-mus18

    authors: Beydoun SR

    更新日期:1998-06-01 00:00:00

  • Neurodevelopmental, behavioral, and emotional symptoms common in Duchenne muscular dystrophy.

    abstract:INTRODUCTION:We studied neurodevelopmental and behavioral/emotional symptoms in patients with Duchenne muscular dystrophy (DMD). METHODS:Retrospective case series of neurodevelopmental and behavioral/emotional symptoms obtained through review of systems of 700 DMD patients in relation to dystrophin gene mutations. RE...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.26803

    authors: Darmahkasih AJ,Rybalsky I,Tian C,Shellenbarger KC,Horn PS,Lambert JT,Wong BL

    更新日期:2020-04-01 00:00:00

  • A syndrome of concurrent central and peripheral nervous system involvement due to Epstein-Barr virus infection.

    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

    authors: Morgenlander JC

    更新日期:1996-08-01 00:00:00

  • Heterogeneity of muscle sizes in the lower limbs of children with cerebral palsy.

    abstract:INTRODUCTION:Cerebral palsy (CP) is associated with reduced muscle volumes, but previous studies have reported deficits in only a small number of muscles. The extent of volume deficits across lower limb muscles is not known. This study presents an imaging-based assessment of muscle volume and length deficits in 35 lowe...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.24972

    authors: Handsfield GG,Meyer CH,Abel MF,Blemker SS

    更新日期:2016-06-01 00:00:00

  • Maximal isometric muscle strength values obtained By hand-held dynamometry in children between 6 and 15 years of age.

    abstract:INTRODUCTION:In this study we aimed to determine the maximal isometric muscle strength of a healthy, normal-weight, pediatric population between 6 and 15 years of age using hand-held dynamometry to establish strength reference values. The secondary objective was determining the relationship between strength and anthrop...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.25180

    authors: Escobar RG,Munoz KT,Dominguez A,Banados P,Bravo MJ

    更新日期:2017-01-01 00:00:00

  • Chorea-acanthocytosis presenting as motor neuron disease.

    abstract:INTRODUCTION:Chorea-acanthocytosis (ChAc) is a rare autosomal recessive disease characterized by involuntary movements, seizures, cognitive changes, myopathy, and axonal neuropathy. METHODS:We report a patient who presented with gait impairment and dysarthria. Clinical and neurophysiological assessment disclosed upper...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.22269

    authors: Neutel D,Miltenberger-Miltenyi G,Silva I,de Carvalho M

    更新日期:2012-02-01 00:00:00

  • Effects of mexiletine on hyperexcitability in sporadic amyotrophic lateral sclerosis: Preliminary findings from a small phase II randomized controlled trial.

    abstract:BACKGROUND:To collect preliminary data on the effects of mexiletine on cortical and axonal hyperexcitability in sporadic amyotrophic lateral sclerosis (ALS) in a phase 2 double-blind randomized controlled trial. METHODS:Twenty ALS subjects were randomized to placebo and mexiletine 300 or 600 mg daily for 4 wk and asse...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.27146

    authors: Weiss MD,Macklin EA,McIlduff CE,Vucic S,Wainger BJ,Kiernan MC,Goutman SA,Goyal NA,Rutkove SB,Ladha SS,Chen IA,Harms MB,Brannagan TH,Lacomis D,Zivkovic S,Ma M,Wang LH,Simmons Z,Rivner MH,Shefner JM,Cudkowicz ME,A

    更新日期:2020-12-19 00:00:00

  • Soleus-specific myopathy induced by passive stretching under local tetanus.

    abstract::Twenty-four adult albino rats were injected with tetanus toxin into the right gastrocnemius muscle and then subjected to sustained dorsiflexion of the right ankle joint for 2 to 14 days. Histologic examinations of the soleus after this procedure showed myopathic changes, characterized by variations in fiber diameters,...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880131007

    authors: Mizuno Y,Chou SM

    更新日期:1990-10-01 00:00:00

  • Action potential shape of rabbit masseter motor units and jaw angle.

    abstract::Action potentials of rabbit masseter motor units (n = 42) were registered at different jaw angles to examine whether the shape of the action potential is related to length of muscle fibers in motor units and depends on the intramuscular location of the motor unit. Twitches were elicited by stimulating motoneurons in t...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.1182

    authors: Turkawski SJ,van Eijden TM

    更新日期:2001-11-01 00:00:00

  • Needlestick injuries among electromyographers.

    abstract::The objective of this study was to determine the self-reported prevalence of needlestick injuries among practicing electromyographers. In January 2008, an anonymous electronic survey was sent to all active members of the American Association for Neuromuscular and Electrodiagnostic Medicine (AANEM) who provided e-mail ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.21118

    authors: Mateen FJ,Grant IA,Sorenson EJ

    更新日期:2008-12-01 00:00:00

  • Comparison of acoustic and electrical signals from erectores spinae muscles.

    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

    authors: Stokes IA,Moffroid MS,Rush S,Haugh LD

    更新日期:1988-04-01 00:00:00

  • Short-term aerobic training response in chronic myopathies.

    abstract::We have previously demonstrated that patients with mitochondrial myopathies can benefit from short-term aerobic exercise training. In this study, we compared the responses to short-term aerobic training of patients with mitochondrial myopathies, patients with nonmetabolic myopathies, and sedentary normal subjects. Tra...

    journal_title:Muscle & nerve

    pub_type: 临床试验,杂志文章

    doi:10.1002/(sici)1097-4598(199909)22:9<1239::aid-mus1

    authors: Taivassalo T,De Stefano N,Chen J,Karpati G,Arnold DL,Argov Z

    更新日期:1999-09-01 00:00:00

  • Effects of hyperbaric exposure with high oxygen concentration on glucose and insulin levels and skeletal muscle-fiber properties in diabetic rats.

    abstract::The effects of hyperbaric exposure with high oxygen concentration on glucose and insulin levels and skeletal muscle-fiber properties were investigated in type 2 diabetic Goto-Kakizaki rats. Five-week-old rats were exposed to a hyperbaric environment (1.25 atmospheric pressure) with a high oxygen concentration (36%) fo...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.20692

    authors: Yasuda K,Adachi T,Gu N,Matsumoto A,Matsunaga T,Tsujimoto G,Tsuda K,Ishihara A

    更新日期:2007-03-01 00:00:00

  • The effect of paired stimuli on blink reflex latencies in normal subjects.

    abstract:INTRODUCTION:In this study we assessed the effect of paired stimuli on the latencies and amplitudes of the blink reflex. METHODS:Blink reflexes were performed with single and paired (5-ms interstimulus interval) stimuli in 47 patients. The changes in latencies between paired and single stimuli were calculated. RESULT...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.22034

    authors: Rubin DI,Dimberg EL,Kennelly KD

    更新日期:2011-08-01 00:00:00

  • Isolated necrotizing myopathy associated with ANTI-PL12 antibody.

    abstract:INTRODUCTION:Immune-mediated myopathies are a heterogeneous group of chronic autoimmune disorders. Autoantibodies associated with this disease complex are classified into myositis-associated and myositis-specific. Anti-tRNA synthetase antibodies are the most well known of the myositis-specific antibodies. Previous repo...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.23383

    authors: Mehndiratta P,Mehta S,Manjila SV,Kammer GM,Cohen ML,Preston DC

    更新日期:2012-08-01 00:00:00

  • Patterns of clinical and electrodiagnostic abnormalities in early amyotrophic lateral sclerosis.

    abstract:INTRODUCTION:The distribution of clinical and neurophysiological abnormalities in patients with early amyotrophic lateral sclerosis (ALS) was investigated in an attempt to delineate patterns of disease spread. METHODS:Clinical and electrodiagnostic data were collected from 150 ALS patients and analyzed based on the cl...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.24244

    authors: Simon NG,Lomen-Hoerth C,Kiernan MC

    更新日期:2014-12-01 00:00:00

  • Protein degradation in cultured skeletal muscle from Duchenne muscular dystrophy patients.

    abstract::The loss of contractile protein in Duchenne muscular dystrophy could result from low rates of synthesis, abnormally high rates of protein degradation, or a combination of both. We measured overall protein degradation rates in cultured human muscle cells obtained at biopsy from patients with Duchenne dystrophy or vario...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880080312

    authors: Neville HE,Harrold S

    更新日期:1985-03-01 00:00:00

  • Carnitine stimulation of pyruvate dehydrogenase complex (PDHC) in isolated human skeletal muscle mitochondria.

    abstract::L-carnitine stimulated CO2 production from 1-14C pyruvate in mitochondria from human skeletal muscle nearly twofold. A comparable increase in the pyruvate dehydrogenase complex (PDHC) activity was seen. Moreover, in the presence of L-carnitine and at pyruvate concentration greater than 0.25 mM, this effect was associa...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.880110708

    authors: Uziel G,Garavaglia B,Di Donato S

    更新日期:1988-07-01 00:00:00

  • Positive sharp wave origin: evidence supporting the electrode initiation hypothesis.

    abstract::This investigation analyzes the temporal characteristics of maximal depolarization times for three waveforms: end-plate spikes, fibrillation potentials, and positive sharp waves (PSWs) to provide support for the electrode initiation hypothesis of PSW induction. The maximal depolarization times for PSWs are documented ...

    journal_title:Muscle & nerve

    pub_type: 杂志文章

    doi:10.1002/mus.20819

    authors: Dumitru D,Santa Maria DL

    更新日期:2007-09-01 00:00:00