Messungen des Membranpotentials einzelner quergestreifter Muskelzellen bei Myotonia congenita (Thomsen)

Riecker, G; Dobbelstein, H; Roehl, D; Hd, Bolte

doi:10.1007/bf01486678

Cited by 21 publications

(11 citation statements)

References 11 publications

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“…This is consistent with findings in the goat (2) and in human myotonia congenita (27). Moreover, the higher-than-normal intracellular potassium concentration of myotonic fibers (see Results) and their lower chloride permeability (see below) is consistent with a higher resting potential in myotonic fibers.…”

Section: Introductionsupporting

confidence: 90%

“…METHODS Normal volunteers. Our normals are 13 males aged [21][22][23][24][25][26][27][28][29][30][31][32][33] The biopsy was placed in a thermos bottle containing previously gassed (95% 02, 5% C02) physiological salt solution at a temperature of from 35 to 38°C, for transportation to the laboratory. Each biopsy was dissected, as previously described (1), so that one or more preparations were used for electrophysiological studies and two to seven preparations for measurement of ion content, ion flux, or extracellular space.…”

Section: Introductionmentioning

confidence: 99%

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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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Section: Introductionsupporting

confidence: 90%

Section: Introductionmentioning

confidence: 99%

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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“…Observations have been made on the resting membrane potential and the presence or absence of 'background' electrical activity; unfortunately, however, the results have differed in the three studies. Thus a fall of resting potential in myotonic fibres was found by Hofmann, Alston, and Rowe (1966) but not by Riecker, Dobbelstein, Rohl, and Bolte (1964) or by Norris (1962;see, however, Discussion).…”

Section: Muscular Dystrophy Laboratories Newcastle General Hospitalmentioning

confidence: 79%

“…On the other hand repetitive depolarization potentials in myotonic fibres were noted by Norris (1962) but not by Riecker et al (1964). In their initial study Hofmann and Rowe (1966) were also unable to detect background activity though it is described in their subsequent report (Hofmann et al, 1966).…”

Section: Muscular Dystrophy Laboratories Newcastle General Hospitalmentioning

confidence: 91%

The electrical properties of muscle fiber membranes in dystrophia myotonica and myotonia congenita.

McComas¹,

Mrozek²

1968

Journal of Neurology, Neurosurgery & Psychiatry

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“…In the early stage of potassium deficiency Em increases slightly, as it does in rats [5] and in humans [28], but after further loss of potassium the measured Em falls to -55 mV, far below the calculated Ediff of -95 mV (see Table 2). To account for this discrepancy, PNa/PK must be about 0.08, eight times the normal value [5], and the Na-K pump must be inactive despite a very high internal sodium level, which would ordinarily produce a large E,,,,.…”

Section: Em Resting Membrane Potentialmentioning

confidence: 96%

Periodic paralysis and the sodium‐potassium pump

Layzer

1982

Annals of Neurology

116

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Analysis of the pathophysiology of hypokalemic paralysis, as it occurs in barium poisoning, chronic potassium deficiency, and thyrotoxicosis, suggests that these disorders may have a similar mechanism. An increased ratio of muscle sodium permeability to potassium permeability reduces the ionic diffusion potential, while the resting membrane potential is sustained by an increase of Na-K pump electrogenesis. The result is that potassium entry (the sum of active and passive influx) exceeds potassium efflux; this causes a large shift of extracellular potassium into muscle until the Na-K pump turns off, leading to depolarization and paralysis. The primary defect in familial hypokalemic periodic paralysis, as in the example of barium poisoning, may be a marked reduction of muscle permeability to potassium.

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Messungen des Membranpotentials einzelner quergestreifter Muskelzellen bei Myotonia congenita (Thomsen)

Cited by 21 publications

References 11 publications

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

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

The electrical properties of muscle fiber membranes in dystrophia myotonica and myotonia congenita.

Periodic paralysis and the sodium‐potassium pump

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