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  • 11
    Electronic Resource
    Electronic Resource
    Springer
    Journal of muscle research and cell motility 4 (1983), S. 177-189 
    ISSN: 1573-2657
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary The force-velocity (T-V) relation and the force-extension (T 1) relation from single fibres isolated from the muscle tibialis anterior of the frog were determined at preset times during the rise of tension and the plateau of an isometric tetanus. During the rise of an isometric tetanus the value of V0 (the velocity of shortening at zero load) remained constant, whereas both the forceT exerted during shortening at a given velocity lower than V0 and the slope of the instantaneousT 1 curve increased with time after the beginning of the stimulus volley. However, while the value ofT (a measure of the level of activation) attained its final value much before the isometric tension attained the plateau, the slope of theT 1 curve (the fibre stiffness, a measure of the number of attached crossbridges) increased throughout the course of tension development. In six muscle fibres, at 2.25µm sarcomere length and at about 4° C, at a time during the tetanus rise whenT had attained 81% of its final value, the fibre stiffness and the isometric tension had risen, respectively, to 50% and to 38% of the values attained at the tetanus plateau. Later, whenT had already attained 99% of its final value, the fibre stiffness and the isometric tension had risen, respectively, to 80% and to 76% of their plateau values. In the same muscle fibres, the average value for the amount of step release required to drop the plateau tetanic tension to zero was only 8.62 nm (observed) or 6.16 nm (extrapolated from the linear part of theT 1 curves) per half sarcomere. It is concluded that the large delay in the development of the isometric tension with respect to the development ofT, and therefore with respect to the development of the wholeT-V relation, cannot be attributed to the passive series compliance of the muscle fibres. It is likely that this delay is due to a specific event in the contractile process.
    Type of Medium: Electronic Resource
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  • 12
    Electronic Resource
    Electronic Resource
    Springer
    Journal of muscle research and cell motility 5 (1984), S. 503-513 
    ISSN: 1573-2657
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary The maximum velocity of shortening (V max) was determined at preset times during the development and the plateau of isometric tetani in single fibres isolated from the tibialis anterior muscle of the frog. Experiments were performed at low temperature (3.6–6° C) and at about 2.25 µm sarcomere length. The controlled velocity release method was used.V max was measured by determining the lowest velocity of release required to keep the tension at zero. Extreme care was taken in dissection and mounting of the fibres in order to make the passive series compliance very small. The value ofV max at the end of the latent period for the development of isometric tension (at 4.5° C about 10 ms after the beginning of the stimulus volley) was already the same as later during either the tension rise or at the plateau of isometric tetani. These results show that the value ofV max of intact fibres is independent of time and activation subsequent to the latent period, and suggest that the cycling rate of the crossbridges may thus attain its steady-state value just at the end of the isometric latent period.
    Type of Medium: Electronic Resource
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  • 13
    ISSN: 1590-3478
    Keywords: Key words Myotonic dystrophy ; Coenzyme Q10 ; CTG ; Lactate threshold
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract An impairment of mitochondrial function may contribute to the pathophysiology of myotonic dystrophy (MyD). Coenzyme Q10 (CoQ10) deficiency has been previously observed, even if in an restricted sample of patients. The aim of this investigation was to obtain more information about coenzyme Q10 and its relationships to the aerobic metabolism in a group of MyD patients. Serum CoQ10 appeared significantly reduced with respect to normal controls: 0.93±0.22 vs. 1.58±0.28 μg/ml (p〈0.05). Moreover, the results demonstrated an inverse tendency between CoQ10 levels and the CTG expansion degree. Basal blood lactate levels were significantly higher than controls (p〈0.05). A borderline inverse correlation between CoQ10 and lactate, corresponding to lactate threshold, was found. These data suggest a possible role of CoQ10 in the pathogenesis of MyD, which may be mediated by mechanisms of cellular damage common to the oxidative pathway. Therapeutic strategies may be devised by virtue of this rationale.
    Type of Medium: Electronic Resource
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  • 14
    ISSN: 1590-3478
    Keywords: Key words MELAS ; Idebenone ; Riboflavin ; Therapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report a patient with MELAS treated for 24 months with idebenone and riboflavin, during which no stroke-like episodes occured. Moreover neurological symptoms clearly improved, and a recovery of brain MRI and EEG abnormalities was observed. We conclude that the combined treatment with idebenone and riboflavin may restore the metabolic impairment in MELAS, possibly improving the long-term prognosis in these patients.
    Type of Medium: Electronic Resource
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