Neuropathological study of neuronal abnormalities showing αB crystallin expression

α b -晶体蛋白（αB-C）是α-晶体蛋白的一个亚基，是脊椎动物晶状体的主要蛋白成分，αB-C在晶状体外组织中有表达。在正常大脑中，少突胶质细胞和星形胶质细胞被抗α b - c抗体染色，而神经元则没有。由于先前使用该抗体的大多数研究主要针对肿胀神经元，因此我们研究了αB-C在各种神经系统疾病的神经元异常中的表达。我们检查了73例29种神经系统疾病患者和4例非神经系统疾病患者。我们从所有患者身上获得了4 μm石蜡包埋的脊髓切片，以及神经系统疾病涉及的一些大脑区域的切片。每个切片采用常规染色法和亲和素-生物素复合物（ABC）法（Vectastain精英，Vector），使用抗α b - c抗体（Hiroshi Mori博士赠送）进行染色。抗αB-C抗体是将αB-C羧基末端合成的多肽注射到家兔体内制备的（Tamaoka et al, 1995）。对αB-C的频繁免疫反应包括皮质基底变性（CBD）和匹克病、皮质路易体、一种新型家族性帕金森病（Mizutani et al ., 1998）和3例中2例伴有假性肥大的下橄榄肾。脑梗死周围部分神经元偶见免疫反应。罕见的免疫反应性包括肌萎缩性侧索硬化症（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运动神经元。在脊髓中，αB-C免疫染色与疾病种类、αB-C免疫染色与组织病理病变分布无一致关系。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性。αB-C阴性免疫反应包括匹克小体、脑干路易小体、阿尔茨海默氏神经原纤维缠结和中枢染色质溶解神经元。结果表明，αB-C对异常神经元的免疫染色具有选择性，该染色可用于检测球化神经元和皮质路易小体，并可用于区分球化神经元与匹克小体和轴突损伤引起的中枢染色质溶解神经元。虽然在其他各种组织病理病变中也观察到神经元免疫反应性，但这种异常很少见，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