Shalev R S, Shalev O, Amir N, Porat S, Fawlewski De Leon G
J Neurol Sci. 1984 Mar;63(3):325-30. doi: 10.1016/0022-510x(84)90155-2.
Because the erythrocyte (RBC) in Duchenne's muscular dystrophy (DMD) is thought to be a suitable experimental paradigm for the sarcolemma, the RBC membrane-bound enzyme (Ca2+ + Mg2+)-ATPase has been investigated as to its relevance to abnormalities of calcium metabolism in DMD muscle. In this study, RBC (Ca2+ + Mg2+)-ATPase activity, intracellular calcium and potassium contents and complete hemogram were examined in 10 DMD patients and 16 age-matched controls. (Ca2+ + Mg2+)-ATPase activity was found elevated in the DMD RBC, consistent with reports from previous studies, but no abnormalities in intracellular calcium, potassium or hemograms were detected. It seems that although the (Ca2+ + Mg2+)-ATPase activity is changed, it bears no relevance to calcium homeostasis in DMD RBC. It is inferred that the increase in intramuscular calcium in DMD muscle, which is also found in other neuromuscular diseases, may be a non-specific finding in the diseased muscle and part of the final common pathway leading toward cellular degeneration and death.
由于杜氏肌营养不良症(DMD)中的红细胞(RBC)被认为是肌膜的合适实验范例,因此对红细胞膜结合酶(Ca2 + + Mg2 +)-ATP酶与DMD肌肉钙代谢异常的相关性进行了研究。在本研究中,检测了10名DMD患者和16名年龄匹配的对照者的红细胞(Ca2 + + Mg2 +)-ATP酶活性、细胞内钙和钾含量以及全血细胞计数。发现DMD红细胞中的(Ca2 + + Mg2 +)-ATP酶活性升高,这与先前研究的报告一致,但未检测到细胞内钙、钾或血细胞计数的异常。似乎尽管(Ca2 + + Mg2 +)-ATP酶活性发生了变化,但它与DMD红细胞中的钙稳态无关。据推测,DMD肌肉中肌内钙的增加,在其他神经肌肉疾病中也有发现,可能是患病肌肉中的非特异性表现,并且是导致细胞变性和死亡的最终共同途径的一部分。