Yarom R, Muhlrad A, Hodges S, Robin G C
Lab Invest. 1980 Sep;43(3):208-16.
The fundamental similarity between platelets and muscle, suggested the possiblity of a shared defect in idiopathic scoliosis, a genetic disease with lateral deformity of the spine in which there is an elevation of calicum concentration in muscles and platelets. A variety of platelet tests revealed the following abnormalities: (1) Electron microscopic x-ray analysis and x-ray fluorescence spectrometry showed a 2- to 3-fold increase in calcium and phosphorus in whole cells and in individual dense bodies. (2) Electron microscopy morphometry revealed an increase in electron-opaque bodies in air-dried cells; granules and microtubules were unchanged. There were more large cells and membranous complexes. (3) Aggregations with epinephrine and ADP were depressed in some patients. (4) Proteins (total and contractile) and myosin. ATPase activity in centrifuged fractions of platelets were decreased in the cytosol and increased in the fraction containing membranes and granules. The correlated findings suggest that platelets in idiopathic scoliosis have a mild calcium transport defect related to membrane and/or contractile protein metabolism. This investigation also shows that platelets may be used to advantage in diagnosis and research of muscle diseases.
血小板与肌肉之间的基本相似性,提示了特发性脊柱侧凸存在共同缺陷的可能性。特发性脊柱侧凸是一种具有脊柱侧方畸形的遗传性疾病,其肌肉和血小板中的钙浓度会升高。一系列血小板检测揭示了以下异常情况:(1)电子显微镜X射线分析和X射线荧光光谱法显示,全细胞及单个致密小体中的钙和磷含量增加了2至3倍。(2)电子显微镜形态计量学显示,空气干燥细胞中电子不透明体增加;颗粒和微管未发生变化。大细胞和膜复合物增多。(3)部分患者对肾上腺素和ADP的聚集反应受到抑制。(4)血小板离心分离物中的蛋白质(总蛋白和收缩蛋白)及肌球蛋白ATP酶活性,在细胞溶质中降低,而在含有膜和颗粒的分离物中升高。这些相关发现表明,特发性脊柱侧凸患者的血小板存在与膜和/或收缩蛋白代谢相关的轻度钙转运缺陷。这项研究还表明,血小板在肌肉疾病的诊断和研究中可能具有优势。