神経筋疾患の phosphorylase isozyme : 筋成熟過程の阻害に関する研究
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As to the pathogenesis of neuromuscular disease, it is very important to clarify the mechanism by which the muscle development and differentiation is impaired or inhibited to a standstill. The presence of phosphorylase activity in cultured muscle cells or regenerating muscle fibers from patients with McArdle disease and its loss during fiber maturity, appears to be extremely iinportant. In order to gain some insight on the relationship between muscle development imd enzyme content, normal development and denervation change of rat muscle fibers were studied, with reference to clinical cases of neuromuscular diseases, using phosphorylase isozymes as markers for differentiation. Normal rat gastrocnemius muscle specimen revealed two bands of fetal type and muscle type phosphorylase isozymes until the third week after birth. After the fourth week, only adult muscle type phosphorylase isozyme was detected. In the rat gastrocnemius muscle which was denervated on the 15th day after birth, obtained 14 days after denervation, fetal type phosphorylase isozyme was retained. Differentiation of phosphorylase isozyme was thus shown to depend on "Trophic" influences of the nerves. In clinical cases, fetal type phosphorylase was detected in the muscle specimens from two cases of Infantile spinal muscular atrophy, a case of Kugerberg-Welander disease, two cases of congenital myopathy, and a case of myotonic dystrophy. Only adult muscle type phosphorylase was detected in 14 other cases. In the muscle which possess fetal type phosphorylase, the impairment of muscle development by denervation or others was suspected.
- 神戸大学の論文
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- 神経筋疾患の phosphorylase isozyme : 筋成熟過程の阻害に関する研究
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