A new mutation in the regulatory domain of the myophosphorylase gene affecting protein dimer contact.

abstract：:Cardiotrophin-1 (CT-1) has potent survival-promoting effects on motor neurons in vitro and in vivo and may be effective in treating motor neuron diseases (MND). We investigated the effects of CT-1 treatment in wobbler mouse MND. Wobbler mice were randomly assigned to receive subcutaneously injected CT-1 (1 mg/kg, n = ...

journal_title：Muscle & nerve

authors： Mitsumoto H,Klinkosz B,Pioro EP,Tsuzaka K,Ishiyama T,O'Leary RM,Pennica D

更新日期：2001-06-01 00:00:00

abstract：:Many patients with the limb-girdle variant of congenital myasthenic syndrome (CMS) possess mutations in the human Dok-7 gene (DOK7). We identified six unrelated CMS patients with DOK7 mutations. Two patients, one mildly and the other moderately affected, were homozygous for the previously described 1263insC mutation. ...

journal_title：Muscle & nerve

authors： Anderson JA,Ng JJ,Bowe C,McDonald C,Richman DP,Wollmann RL,Maselli RA

更新日期：2008-04-01 00:00:00

abstract：:In an attempt to determine the relative importance of the nerve cell body and of the axon in initiating and controlling axonal regeneration, nerve cell bodies were irradiated and the ability of the distal axon to sprout was examined. Mice were subjected to either 25 or 50 Gray (Gy) of x-irradiation localized to the lu...

journal_title：Muscle & nerve

authors： Pamphlett RS

更新日期：1988-05-01 00:00:00

abstract：:The myogenic regulatory factor MyoD plays an important role in embryonic and adult skeletal muscle growth. Even though it is best known as a marker for activated satellite cells, it is also expressed in myonuclei, and its expression can be induced by a variety of different conditions. Several model systems have been u...

journal_title：Muscle & nerve

authors： Legerlotz K,Smith HK

更新日期：2008-09-01 00:00:00

abstract：:Evaluation of the precise molecular dynamics of endplate maintenance and reorganization has been limited by the lack of available in vitro preparations. We describe an organ culture preparation of mouse diaphragm muscle which permits long-term maintenance of muscle viability. Spontaneous fibrillations, increased level...

journal_title：Muscle & nerve

authors： Wetzel DM,Salpeter MM

更新日期：1991-10-01 00:00:00

abstract：:A treatment has been developed to alleviate muscle weakness in murine dystrophy. Cultured myoblasts from genetically normal mouse embryos were injected into the right soleus of 20-day-old normal or dystrophic mice. Hosts and donors were immunocompatible but exhibited different genotype markers. Donor cells produced GP...

journal_title：Muscle & nerve

authors： Law PK,Goodwin TG,Wang MG

更新日期：1988-06-01 00:00:00

abstract：INTRODUCTION:In mitochondrial disease, it is likely that energy substrate depletion leads to paralysis of ATPase-dependent pumps, resulting in membrane depolarization. Axonal depolarization has been demonstrated in a crisis, but not in the resting state. We, therefore, stressed axons using ischemia to see if this would...

journal_title：Muscle & nerve

authors： Ng K,Kumar KR,Sue C,Burke D

更新日期：2013-05-01 00:00:00

abstract：INTRODUCTION:Transitioning from adolescence to adulthood can be problematic for individuals with rare disabilities such as muscular dystrophy (MD). METHODS:We identified a cohort of 220 individuals with MD and 440 matched comparison individuals and measured emergency room (ER) and inpatient (IP) encounters for the yea...

journal_title：Muscle & nerve

authors： Mann JR,Royer JA,Mcdermott S,Hardin JW,Ozturk O,Street N

更新日期：2015-11-01 00:00:00

abstract：:Muscle weakness, studied in 4 patients with multiple sclerosis (MS), was compared with values from normal subjects. Twitch occlusion showed that normal subjects could activate their muscles maximally, but patients rarely achieved greater than 60% activation. In both groups, motoneuron firing rates increased linearly w...

journal_title：Muscle & nerve

authors： Rice CL,Vollmer TL,Bigland-Ritchie B

更新日期：1992-10-01 00:00:00

abstract：:The role of motor innervation and muscle tension in the posthatching maturation of the slow-tonic anterior latissimus dorsi (ALD) muscle of the chicken has been investigated. Modification of the muscle tension was obtained either by maintaining ALD in a shortened state or by stretching, after or without denervation. I...

journal_title：Muscle & nerve

authors： Gardahaut MF,Rouaud TP,Renaud D,Khaskiye A,Le Douarin GH

更新日期：1989-11-01 00:00:00

abstract：:Electrical stimulation was applied daily for 20 minutes to denervated rabbit extensor digitorum longus muscle. One group was stimulated with short tetani, another with 1-Hz frequency, using isometric contractions for both. Tetanic stimulation induced severe fibrosis and is harmful to denervated muscle. One Hertz stimu...

journal_title：Muscle & nerve

authors： Nix WA,Dahm M

更新日期：1987-02-01 00:00:00

abstract：:To clarify the molecular nature of the pathogenesis in X-linked Emery-Dreifuss muscular dystrophy (EDMD), we monitored the expression of 2400 genes in control and EDMD fibroblasts by using complementary DNA (cDNA) microarray techniques. A total of 60 genes whose expression was altered in EDMD fibroblasts when compared...

journal_title：Muscle & nerve

authors： Tsukahara T,Tsujino S,Arahata K

更新日期：2002-06-01 00:00:00

abstract：:Several studies have suggested the presence of central nervous system involvement manifesting as cognitive impairment in diseases traditionally confined to the peripheral nervous system. The aim of this review is to highlight the character of clinical, genetic, neurofunctional, cognitive, and psychiatric deficits in n...

journal_title：Muscle & nerve

authors： D'Angelo MG,Bresolin N

更新日期：2006-07-01 00:00:00

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

authors： Kalita J,Kumar M,Misra UK

更新日期：2018-05-01 00:00:00

abstract：INTRODUCTION:Muscle changes in patients with diabetes and lower leg pain due to chronic exertional compartment syndrome (CECS) were investigated before and after fasciotomy. METHODS:The tibialis anterior muscle was analyzed with histochemical and morphological techniques in 7 patients with diabetes and CECS before fas...

journal_title：Muscle & nerve

authors： Edmundsson DS,Toolanen GL,Stål PS

更新日期：2018-02-01 00:00:00

abstract：:Electromyographic (EMG) recordings may serve an important role in predicting torque during repetitive activation of paralyzed muscle. We compared the initial M-wave to the subsequent M-waves of the same train under fatigued and recovered conditions in the paralyzed human soleus muscle. Sixteen individuals with chronic...

journal_title：Muscle & nerve

authors： Chang YJ,Shields RK

更新日期：2002-11-01 00:00:00

abstract：INTRODUCTION:Surface electromyography (SEMG) allows objective assessment and guides selection of appropriate treatment in focal hand dystonia (FHD). METHODS:Sixteen-channel SEMG obtained during different phases of a writing task was used to study timing, activation patterns, and spread of muscle contractions in FHD co...

journal_title：Muscle & nerve

authors： Sivadasan A,Sanjay M,Alexander M,Devasahayam SR,Srinivasa BK

更新日期：2013-09-01 00:00:00

abstract：:Reinnervation of a free muscle graft by nerves from an adjacent intact muscle is called muscular neurotization. This paper investigates the mechanisms and stimuli responsible for muscular neurotization in the rat. Sternohyoid or sternomastoid muscles were transplanted as free muscle grafts to the ventral surface of an...

journal_title：Muscle & nerve

authors： Must R

更新日期：1987-07-01 00:00:00

abstract：INTRODUCTION:The aim of this study was to evaluate the ultrasound (US) morphological changes of sural nerves (SNs) of uremic patients on hemodialysis. METHODS:Sixty-six SNs in 33 uremic patients were examined by 22-MHZ high-frequency US and routine nerve conduction studies (NCS), and 76 SNs in 38 controls were also ex...

journal_title：Muscle & nerve

authors： Wei M,Zhu J,Wang N,Xue Q,Liu F,Hu B,Fang J,Zhang Y

更新日期：2012-12-01 00:00:00

abstract：INTRODUCTION:Myasthenia gravis (MG) is a B-cell-mediated autoimmune disease. B-cell-activating factor (BAFF) is a major factor in B-cell development and activation. In this study we investigated serum BAFF levels in MG patients. METHODS:We compared the serum BAFF levels of 20 MG patients with gender-matched healthy co...

journal_title：Muscle & nerve

authors： Kang SY,Kang CH,Lee KH

更新日期：2016-12-01 00:00:00

abstract：:Genetically homozygous line 413 dystrophic chickens were given twice-daily intraperitoneal injections of solutions containing branched-chain amino acids (BCCA-leucine, valine, isoleucine) either alone or in combination; and their alpha-ketoacid analogs (alpha-ketoisocaproic and alpha-ketoisovaleric acids). Another tri...

journal_title：Muscle & nerve

authors： Hudecki MS,Pollina CM,Heffner RR

更新日期：1982-07-01 00:00:00

abstract：:We investigated 16 patients with Fabry's disease (eight hemizygous men and eight heterozygous women) in one family. We used constant current perception threshold (CPT) testing, which evaluated three major sensory nerve fiber populations, to assess subjective complaints of pain and paresthesias. We also examined clinic...

journal_title：Muscle & nerve

authors： Ro LS,Chen ST,Tang LM,Hsu WC,Chang HS,Huang CC

更新日期：1999-11-01 00:00:00

abstract：:The model of chronic low-frequency stimulation for the study of muscle plasticity was developed over 30 years ago. This protocol leads to a transformation of fast, fatigable muscles toward slower, fatigue-resistant ones. It involves qualitative and quantitative changes of all elements of the muscle fiber studied so fa...

journal_title：Muscle & nerve

authors： Pette D,Vrbová G

更新日期：1999-06-01 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

authors： Keesey JC

更新日期：1989-08-01 00:00:00

abstract：:Masseter muscle biopsies were obtained from nine patients undergoing orthognatic surgery or surgery for parotid tumors. A detailed enzyme-histochemical and immunocytochemical study of these muscles was performed using antibodies specific to the various isozymes of the myosin heavy chain (MHC) in order to identify the ...

journal_title：Muscle & nerve

authors： Butler-Browne GS,Eriksson PO,Laurent C,Thornell LE

更新日期：1988-06-01 00:00:00

abstract：INTRODUCTION:In this study, we analyzed the crosstalk in mechanomyographic (MMG) signals generated by the extensor digitorum (ED), extensor carpi ulnaris (ECU), and flexor carpi ulnaris (FCU) muscles of the forearm during wrist flexion (WF) and extension (WE) and radial (RD) and ulnar (UD) deviations. METHODS:Twenty r...

journal_title：Muscle & nerve

authors： Islam A,Sundaraj K,Ahmad RB,Sundaraj S,Ahamed NU,Ali MA

更新日期：2015-06-01 00:00:00

abstract：INTRODUCTION:In this study we compare the ultrasound features in the median nerve in patients with different types of Charcot-Marie-Tooth (CMT) disease and hereditary neuropathies with liability to pressure palsies (HNPP) as a typical entrapment neuropathy. METHODS:Median nerve ultrasound and conduction studies were p...

journal_title：Muscle & nerve

authors： Schreiber S,Oldag A,Kornblum C,Kollewe K,Kropf S,Schoenfeld A,Feistner H,Jakubiczka S,Kunz WS,Scherlach C,Tempelmann C,Mawrin C,Dengler R,Schreiber F,Goertler M,Vielhaber S

更新日期：2013-03-01 00:00:00

abstract：INTRODUCTION:Previous studies have reported higher wrist ratios (WR) related to carpal tunnel syndrome (CTS) but have not assessed effect modification by obesity and may have inadequately controlled for confounders. METHODS:Baseline data of a multicenter prospective cohort study were analyzed. CTS was defined by nerve...

journal_title：Muscle & nerve

authors： Thiese MS,Merryweather A,Koric A,Ott U,Wood EM,Kapellusch J,Foster J,Garg A,Deckow-Schaefer G,Tomich S,Kendall R,Drury DL,Wertsch J,Hegmann KT,Wistah Study Team.

更新日期：2017-12-01 00:00:00

abstract：:Twenty patients with neurogenic disorders, polymyositis, or muscular dystrophies were assessed clinically and by CT imaging of limb, limb girdle, and trunk muscles, using a standard protocol. On each side of these patients 26 movements were graded by the MRC scale, and 20 muscles were assessed by CT imaging. The clini...

journal_title：Muscle & nerve

authors： Swash M,Brown MM,Thakkar C

更新日期：1995-07-01 00:00:00

abstract：:Ion channels are complex proteins that span the lipid bilayer of the cell membrane, where they orchestrate the electrical signals necessary for normal function of the central nervous system, peripheral nerve, and both skeletal and cardiac muscle. The role of ion channel defects in the pathogenesis of numerous disorder...

journal_title：Muscle & nerve

authors： Kleopa KA,Barchi RL

更新日期：2002-09-01 00:00:00