UBIQUITIN AND BETA-AMYLOID-PROTEIN IN INCLUSION-BODY MYOSITIS (IBM), FAMILIAL IBM-LIKE DISORDER AND OCULOPHARYNGEAL MUSCULAR-DYSTROPHY - AN IMMUNOCYTOCHEMICAL STUDY
UBIQUITIN AND BETA-AMYLOID-PROTEIN IN INCLUSION-BODY MYOSITIS (IBM), FAMILIAL IBM-LIKE DISORDER AND OCULOPHARYNGEAL MUSCULAR-DYSTROPHY - AN IMMUNOCYTOCHEMICAL STUDY
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DOI：
10.1016/0960-8966(93)90021-b
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发表时间：
1993-01-01
影响因子：
2.8
通讯作者：
FARDEAU, M
中科院分区：
文献类型：
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作者：
LECLERC, A;TOME, FMS;FARDEAU, M
我们使用免疫细胞化学方法在三种神经肌肉疾病患者的肌肉活检组织中鉴定泛素和β-淀粉样蛋白，这些疾病的特征是肌肉纤维中存在边缘空泡：包涵体肌炎(IBM)、家族性IBM样疾病和眼咽肌营养不良(OPMD)。在所有三种疾病中都观察到了抗泛素抗体的标记，但在IBM中很常见，在家族性IBM样疾病中较少见，在OPMD中很少见。这种标记被认为对应于IBM类型细丝(外径16-18 nm)的存在，这种细丝是IBM或家族性IBM样疾病的特征，但不是特异性的，因为它们也可能出现在包括OPMD在内的其他疾病中。在IBM的少数纤维中发现了抗β-淀粉样蛋白抗体的标记，而在其他两种情况下则没有。用这种抗体标记的结构尚未确定。用抗泛素或抗β-淀粉样蛋白抗体标记与酸性磷酸酶活性无关。
We used immunocytochemistry to identify ubiquitin and beta-amyloid-protein in muscle biopsies from patients with three neuromuscular disorders characterized by the presence of rimmed vacuoles in muscle fibres: inclusion body myositis (IBM), familial IBM-like disorder and oculopharyngeal muscular dystrophy (OPMD). Labelling with anti-ubiquitin antibodies was observed in all three diseases, but it was frequent in IBM, less common in familial IBM-like disorder and rare in OPMD. This labelling is thought to correspond to the presence of IBM-type filaments (16-18 nm in external diameter) which are characteristic but not specific for IBM or familial IBM-like disorder, as they may also occur in other diseases including OPMD. Labelling with anti-beta-amyloid-protein antibody was seen in a few fibres in IBM but not in the other two conditions. The structures labelled with this antibody have yet to be determined. Labelling with anti-ubiquitin or anti-beta-amyloid-protein antibodies was not correlated with the presence of acid phosphatase activity.