A study of individual neuro-muscular junctions in myotonia

Hofmann, William W.; Alston, Walter; G, Rowe

doi:10.1016/0013-4694(66)90171-4

Cited by 57 publications

(25 citation statements)

References 44 publications

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“…In the present study we (Ludin, 1967) and in hereditary mouse dystrophy (Kleeman, Partridge, and Glaser, 1961;Lenman, 1963;McComas and Mossawy, 1965 Elmqvist et al (1964) and Hofmann et al (1966). So far as the critical membrane depolarization is concerned, the values obtained by Elmqvist et al (1964) (7-20 mV) were of the same order as those found in the present investigation.…”

Section: Methodssupporting

confidence: 82%

“…During recent years a number of investigations have been concerned with the measurement of the resting membrane potential in human skeletal muscle fibres (Johns, 1960;Elmqvist, Johns, and Thesleff, 1960;Norris, 1962;Creutzfeldt, Abbott, Fowler, and Pearson, 1963;Bolte, Riecker, and Rohl, 1963;Goodgold and Eberstein, 1966;Hofmann, Alston, and Rowe, 1966;Ludin, 1967;Brooks and Hongdalarom, 1968). …”

mentioning

confidence: 99%

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Electrical properties of muscle fibre membranes in man.

McComas¹,

Mrozek²,

Gardner-Medwin³

et al. 1968

Journal of Neurology, Neurosurgery & Psychiatry

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Section: Methodssupporting

confidence: 82%

mentioning

confidence: 99%

Electrical properties of muscle fibre membranes in man.

McComas¹,

Mrozek²,

Gardner-Medwin³

et al. 1968

Journal of Neurology, Neurosurgery & Psychiatry

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“…We, as others (3,4,(12)(13)(14), have found that it can provide useful, reproducible electrophysiological, ion flux, and ion content data.…”

Section: Introductionmentioning

confidence: 73%

Cable parameters, sodium, potassium, chloride, and water content, and potassium efflux in isolated external intercostal muscle of normal volunteers and patients with myotonia congenita

Lipicky¹,

Bryant²,

Salmon³

1971

J. Clin. Invest.

150

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A B S T R A C T In isolated fiber bundles of external intercostal muscle from each of 13 normal volunteers and each of 6 patients with myotonia congenita, some or all of the following were measured: concentrations of Na+, K+, and Cl-, extracellular volume, water content, K+ efflux, fiber size, fiber cable parameters, and fiber resting potentials.Muscle from patients with myotonia congenita differed significantly (0.001 < P< 0.025) with respect to the following mean values (myotonia congenita vs. normal): the membrane resistance was greater (5729 vs. 2619 U. cm2), the internal resistivity was less (75.0 vs. 123.2 Q-cm), the water content was less (788.2 vs. 808.2 ml/kg wet weight), and the mean resting potential was greater (68 vs. 61 mv).No significant differences were found with respect to the following variables: K+ content (73.5 vs. 66.7 mEq/kg wet weight) and the calculated intracellular K+ concentration (215 vs. 191 mEq/liter fiber water), fiber capacitance (5.90 vs. 5.15 Mf/cm2), Na+ content (97.7 vs. 94.1 mEq/kg wet weight), 74.7 mEq/kg wet weight), mannitol extracellular volume (45.1 vs. 46.6 cc/100 g wet weight), and K+ efflux (23.2 vs. 21.5 moles X 10-12 cm-2.sec-1).These abnormalities of skeletal muscle in human myotonia congenita are like those of skeletal muscle in goats with hereditary myotonia. We tentatively conclude that a decreased Cl-permeability accounts for some of the abnormal electrical properties of skeletal muscle in myotonia congenita. We now report findings in isolated external intercostal muscle from patients with myotonia congenita and from normal volunteers. We have compared our human results with similar data we obtained in isolated external intercostal muscle from the goat (1, 2). METHODS Normal volunteers. Our normals are 13 males aged 21-33 yr with no evidence of neuropathy or myopathy. One (R. G.) had diabetes mellitus; another (T. P.) had probable pulmonary sarcoidosis (minimal pulmonary fibrosis on chest X-ray and scalene node biopsy positive for noncaseating granuloma). At the time of muscle biopsy, T. P. had no symptoms and his chest X-ray was clearer (without therapy) than 1 yr earlier. No volunteer had taken any medication for at least 5 days before the external intercostal muscle biopsy except for the patient with diabetes, who received insulin up to and including the day before biopsy. The remaining 11 subjects were healthy and the results of laboratory studies (chest X-ray, electrocardiogram, hemoglobin, hematocrit, white blood cell count and differential, urinalysis, serum urea nitrogen, fasting blood sugar, Wasserman, serum creatinine, serum ions [Na+, K+, Cl-], serum Ca and Mg, total serum proteins, serum alkaline phosphatase, serum glutamic oxalacetic transaminase, and creatinine phosphokinase) were normal. Biopsy techniques. Biopsies of the anterior border of external intercostal muscle (2.5-5 cm in length) were obtained under local anesthesia from the right eighth intercostal space. Preoperative medications were pentobarbital (from 100 to 200 mg), and morphin...

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“…This disease is known to be a systemic genetic disorder inherited as an autosomal dominant trait. Electrophysiological studies have shown that the resting membrane potential is reduced (28,(30)(31)(32)(33) and that the membrane resistance is slightly increased (34). The overshoot of the action potential is not significantly affected (32) or only slightly decreased (33) and, like in normal muscle, the action potential is completely abolished after a 3-min exposure to 1 ,M TTX (7,33).…”

mentioning

confidence: 99%

Sodium Channel and Sodium Pump in Normal and Pathological Muscles from Patients with Myotonic Muscular Dystrophy and Lower Motor Neuron Impairment

Desnuelle¹,

Lombet²,

Serratrice³

et al. 1982

J. Clin. Invest.

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A B S T R A C T Two sodium transport systems have been analyzed in this work: the voltage-sensitive sodium channel and the (Na+, K+) ATPase pump. The sodium channel has been studied using a tritiated derivative of tetrodotoxin; the sodium pump has been studied using tritiated ouabain. Properties of interaction of tritiated tetrodotoxin and of tritiated ouabain with their respective receptors were observed in normal human skeletal muscle and in muscles of patients with myotonic muscular dystrophy and with lower motor neuron impairment.Levels of sodium pump and of sodium channels were measured at different stages of membrane purification. Microsomal fractions of normal human muscle have maximal binding capacities for tetrodotoxin of 230 fmol/mg of protein and of 7.4 pmol/mg of protein for ouabain.Dissociation constant for the complexes formed by the tetrodotoxin derivative and by ouabain with their respective receptors were 0.52 nM and 0.55 ,uM, respectively.In muscles from patients with myotonic muscular dystrophy, the maximal binding capacity for tetrodotoxin, i.e., the number of Na+ channels was found to be very similar to that found for normal muscle.The maximal binding capacity for ouabain, i.e., the number of Na+ pumps was three-to sixfold lower than in normal muscle. Dissociation constants for the com-

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A study of individual neuro-muscular junctions in myotonia

Cited by 57 publications

References 44 publications

Electrical properties of muscle fibre membranes in man.

Electrical properties of muscle fibre membranes in man.

Cable parameters, sodium, potassium, chloride, and water content, and potassium efflux in isolated external intercostal muscle of normal volunteers and patients with myotonia congenita

Sodium Channel and Sodium Pump in Normal and Pathological Muscles from Patients with Myotonic Muscular Dystrophy and Lower Motor Neuron Impairment

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