Vainzof M, Zubrzycka-Gaarn E E, Rapaport D, Passos-Bueno M R, Pavanello R C, Pavanello-Filho I, Zatz M
Departamento de Biologia, Universidade de São Paulo, Brazil.
J Neurol Sci. 1991 Feb;101(2):141-7. doi: 10.1016/0022-510x(91)90038-9.
Dystrophin immunohistochemical studies in muscle from Duchenne patients (DMD) have shown a population of fibers with partial labelling. In order to determine whether this is related to a cross reaction or to the presence of dystrophin. 22 DMD patients were studied immunohistochemically, through the concomitant use of antibodies from the N-terminal and the C-terminal regions of the protein. In 2, the reaction was negative while in 2 others 17 and 25% of fibers were positive with both antibodies. In the remainder, a population of partially stained fibers was seen: 11 were positive with both antibodies and in 7 only with the N-terminal one. Apparently, there is no correlation between the proportion of positive fibers and clinical progression, or the presence and pattern of DNA deletions in the central part of the gene. These observations led us to suggest that some truncated protein, intermediate synthesis or degradation products of dystrophin are present in muscle from Duchenne patients.
对杜氏肌营养不良症（DMD）患者肌肉进行的肌营养不良蛋白免疫组织化学研究显示，有一群纤维呈现部分标记。为了确定这是与交叉反应还是与肌营养不良蛋白的存在有关，对22例DMD患者进行了免疫组织化学研究，通过同时使用来自该蛋白N端和C端区域的抗体。2例患者反应为阴性，另外2例患者中，两种抗体检测均显示17%和25%的纤维呈阳性。其余患者中，可见一群部分染色的纤维：11例患者两种抗体检测均呈阳性，7例患者仅N端抗体检测呈阳性。显然，阳性纤维比例与临床进展之间，或基因中部DNA缺失的存在及模式之间并无关联。这些观察结果使我们推测，杜氏肌营养不良症患者的肌肉中存在一些截短蛋白、肌营养不良蛋白的中间合成或降解产物。
