Neuropathological study of neuronal abnormalities showing αB crystallin expression

α B-晶状体蛋白（αB-C）是脊椎动物透镜的主要蛋白组分α-晶状体蛋白的一个亚基，在晶状体外组织中有表达。在正常脑中，少突胶质细胞和星形胶质细胞被抗αB-C抗体染色，而神经元不被染色。由于以往大多数使用该抗体的研究主要针对气球样神经元，因此我们研究了各种神经系统疾病的神经元异常中的αB-C表达。我们检查了73例29种神经系统疾病和4例非神经系统疾病患者。我们获得了所有患者脊髓的4 μm石蜡包埋切片，以及神经系统疾病涉及的某些脑区的切片。使用抗αB-C抗体（Hiroshi Mori博士赠送），通过常规染色方法和抗生物素蛋白-生物素复合物（ABC）方法（Vectastain elite，Vector）对每个切片进行染色。抗-αB-C抗体是通过注射合成的抗-αB-C抗体， ...更多信息 对α B-C的免疫反应性包括皮质基底节变性（CBD）和皮克病中的气球样神经元、皮质Lewy小体（一种新的家族性帕金森病中的气球样神经元）（Mizutani et al，1998）以及3例中2例伴有假性肥大的下橄榄核的重核。脑梗死灶周围部分神经元偶见免疫反应阳性。罕见的免疫反应性包括肌萎缩侧索硬化症（ALS）、进行性核上性麻痹（PSP）、Machado-Joseph病、包涵体肌炎、多发性硬化和癌性脑膜炎的脊髓前角运动神经元，以及ALS、PSP、肝性脊髓病、包涵体肌炎和隐球菌脑膜炎的脊髓后角神经元。在脊髓中，α B-C免疫染色与疾病种类、组织病理学病变分布之间无一致性关系。在多系统萎缩和CBD的脑桥核神经元中也发现了罕见的免疫反应。αB-C免疫反应阴性的神经元包括Pick小体、脑干Lewy小体、Alzheimer's神经纤维缠结和中央染色质溶解的神经元，表明αB-C免疫反应对异常神经元具有选择性，可用于检测气球样变神经元和皮质Lewy小体，并可用于区分气球样变神经元与Pick小体和轴突损伤引起的中央染色质溶解神经元。虽然在其他各种组织病理学病变中观察到神经元免疫反应性，但这种异常是罕见的，需要进一步的研究来阐明这种神经元免疫反应性的意义。少

αB-crystallin (αB-C) is a subunit of α-crystallin, a major protein component of the vertebrate lens, and αB-C expression has been reported in extra-lenticular tissues. In normal brains, oligodendrocytes and astrocytes are stained with anti-αB-C antibody, but neurons are not. Since most of previous studies using this antibody have primarily dealt with ballooned neurons, we investigated such αB-C expression in neuronal abnormalities of various neurological diseases.We examined 73 patients with 29 kinds of neurological diseases and 4 patients with non-neurological diseases. We obtained 4-μm paraffin embadded sections of the spinal cords from all of the patients, in addition to the sections of some areas of the brains involved by neurological diseases. Each section was stained by the routine staining methods and avidin-biotin complex (ABC) method (Vectastain elite, Vector) using anti-αB-C antibody (a gift from Dr. Hiroshi Mori). The anti-αB-C antibody was made by the injection of a synthet … More ic peptide from the carboxyl terminal of αB-C to rabbits (Tamaoka et al, 1995).Frequent immunoreactivity to αB-C included ballooned neurons in corticobasal degeneration (CBD) and Pick's disease, cortical Lewy bodies, ballooned in a new type of familial Parkinson disease (Mizutani et al, 1998), and renurons of the inferior olives with pseudohypertrophy in 2 out of 3 cases. Occasional immunoreactivity was observed in some neurons around cerebral infarcts. Rare immunoreactivity included the anterior hom motor neurons of the spinal cords in amyotrophic lateral sclerosis (ALS), progressive supranuclear palsy (PSP), Machado-Joseph disease, inclusion body myositis, multiple sclerosis, and carcinomatous meningitis, as well as the posterio hom neurons of the spinal cords in ALS, PSP, hepatic myelopathy, inclusion body myositis, and crytococcal meningitis. In the spinal cords, no consistent relationship was observed between the αB-C immunostaining and the kind of diseases, and between the αB-C immunostaining and the distribution of the histopathological lesions. Rare immunoreactivity was also found in the pontine nuclei neurons of multiple system atrophy and CBD. Negative immunoreactivity to αB-C included Pick bodies, brainstem Lewy bodies, Alzheimer's neurofibrillary tangles, and neurons with central chromatolysis.Our results indicated that αB-C immunostaining to abnormal neurons was selective, and that this staining was useful to detect ballooned neurons and cortical Lewy bodies, and to differentiate balloned neurons from both Pick bodies and neurons with central chromatolysis due to axonal injury. Although neuronal immunoreactivity was observed in other various histopathological lesions, this abnormality was rare, and further studies are needed to clarify the significance of such neuronal immunoreactivity. Less