BUNINA BODIES IN AMYOTROPHIC-LATERAL-SCLEROSIS IMMUNOSTAINED WITH RABBIT ANTI-CYSTATIN-C SERUM
BUNINA BODIES IN AMYOTROPHIC-LATERAL-SCLEROSIS IMMUNOSTAINED WITH RABBIT ANTI-CYSTATIN-C SERUM
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DOI：
10.1016/0304-3940(93)90576-7
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发表时间：
1993-11-12
影响因子：
2.5
通讯作者：
SAKURAI, A
中科院分区：
文献类型：
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作者：
OKAMOTO, K;HIRAI, S;SAKURAI, A
肌萎缩侧索硬化症(ALS)的唯一病理特征是肌萎缩侧索硬化症(ALS)的唯一病理特征是布尼娜小体(Bbs)，即残留的下运动神经元中的小的嗜酸性神经元内包涵体。在ALS患者脊髓的免疫组织化学检查中，我们注意到BBS的抗胱抑素C(CC)血清呈阳性。免疫电子显微镜下，在前角细胞的胞质和树突内可见许多免疫过氧化物酶产物的小沉积。部分免疫过氧化物酶产物呈管状或泡状，未见粗面内质网、线粒体、脂褐素颗粒或细胞核。BBS的存在，特别是在外围地区，是很明显的。在成年猫，CC主要分布在高尔基体的内侧和脊髓前角细胞的溶酶体中。BBS可能表现出与高尔基体有关的未知蛋白物质的异常堆积。
Bunina bodies (BBs), small eosinophilic intraneuronal inclusions in the remaining lower motor neurons, are the only pathologically specific hallmark of amyotrophic lateral sclerosis (ALS). During immunohistochemical examinations of spinal cords of patients with ALS, we noted that BBs were positive for anti-cystatin C (CC) serum. Immunoelectron microscopically, many small deposits of immunoperoxidase products were seen in the cytoplasms and dendrites of the anterior horn cells. Some immunoperoxidase products exhibited a tubular or vesicular pattern, but no rough endoplasmic reticula, mitochondria, lipofuscin granules or nuclei were detected. The presence of BBs, especially in the periphery, was marked. In an adult cat, CC was mainly localized in the medial aspects of Golgi apparatus and in lysosomes in the anterior horn cells of the spinal cord. BBs may reresent an abnormal accumulation of unknown proteinous material associated with the Golgi apparatus.