Degl'Innocenti D, Pieri A, Rosati F, Ramponi G
Department of Biochemical Sciences, University of Florence, Firenze, Italy.
IUBMB Life. 1999 Oct;48(4):391-6. doi: 10.1080/713803541.
Muscular dystrophy is a genetic disease that affects primarily skeletal muscle. The dystrophin absence has been related to the degeneration of muscle fibres. Indirect evidences suggest that oxidative stress may play a role in the pathogenesis of the disease, but the significance and precise extent of this contribution is poorly understood. In this paper we show that Becker Muscular Dystrophy (BMD) and Duchenne Muscular Dystrophy (DMD) skin fibroblasts are more susceptible to H2O2 treatment than are fibroblasts from unaffected persons. In particular, we found that, in growing DMD skin fibroblasts, the oxidative treatment resulted in significantly reduced growing capacity. We also investigated the concentrations of intracellular calcium during H2O2 treatment. The intracellular free calcium concentration increased by 22%, 35%, and 40% in unaffected, BMD, and DMD fibroblasts, respectively. However, the increase of the intracellular free calcium concentration is not related, as previously hypothesized, to a reduction of acylphosphatase concentrations, which seem to be unaffected by the H2O2 treatment, but rather to reduced enzyme activity.
肌肉萎缩症是一种主要影响骨骼肌的遗传性疾病。肌营养不良蛋白的缺失与肌纤维的退化有关。间接证据表明氧化应激可能在该疾病的发病机制中起作用，但这种作用的重要性和确切程度尚不清楚。在本文中，我们表明，与未受影响者的成纤维细胞相比，贝氏肌肉萎缩症（BMD）和杜氏肌肉萎缩症（DMD）的皮肤成纤维细胞对过氧化氢处理更敏感。特别是，我们发现，在生长的DMD皮肤成纤维细胞中，氧化处理导致生长能力显著降低。我们还研究了过氧化氢处理过程中细胞内钙的浓度。未受影响、BMD和DMD成纤维细胞内的游离钙浓度分别增加了22%、35%和40%。然而，细胞内游离钙浓度的增加并不像之前假设的那样与酰基磷酸酶浓度的降低有关，酰基磷酸酶浓度似乎不受过氧化氢处理的影响，而是与酶活性降低有关。
