Foley J, Witte D, Chiu F C, Parysek L M
Department of Anatomy and Cell Biology, University of Cincinnati College of Medicine, Ohio.
Lab Invest. 1994 Aug;71(2):193-9.
Peripherin and neurofilament (NF)-66/alpha-internexin are recently characterized, neuron-specific intermediate filament proteins that are expressed in the developing peripheral nervous system. Peripherin, in particular, is highly enriched in neuronal derivatives of the neural crest. We speculated that these intermediate filament proteins would be expressed in neuroblastoma (NB), a neural crest-derived tumor with many neuronal features.
By use of antibodies specific to peripherin and NF-66/alpha-internexin, we detected these proteins on Western blots of NB tissue extracts and in paraffin sections of NBs.
Western blotting indicated that NB tumor extracts contained immunoreactive proteins that co-migrated with rat peripherin and human NF-66/alpha-internexin from normal tissues, thus establishing the specificity of the antibodies for these proteins in tumors. The antibody specific for peripherin labeled all NBs, including immature NBs, composite ganglioneuroblastomas and ganglioneuromas. In contrast, the NF-66/alpha-internexin antibody labeled only 50% of NBs, and only weakly labeled most ganglioneuroblastomas and ganglioneuromas. Neither antibody labeled other small blue cell tumors such as lymphomas, rhabdomyosarcomas, Wilms' tumors, and Ewing sarcomas.
The specificity of the peripherin labeling of NB and the ability of the peripherin antibody to label the entire spectrum of NBs, including ganglioneuroblastomas and ganglioneuromas, indicate that this intermediate filament protein has potential as a diagnostic marker for these related neural crest neoplasms.
外周蛋白和神经丝（NF）-66/α-中间丝蛋白是最近鉴定出的神经元特异性中间丝蛋白，在发育中的外周神经系统中表达。特别是外周蛋白，在神经嵴的神经元衍生物中高度富集。我们推测这些中间丝蛋白会在神经母细胞瘤（NB）中表达，NB是一种具有许多神经元特征的神经嵴衍生肿瘤。
通过使用针对外周蛋白和NF-66/α-中间丝蛋白的特异性抗体，我们在NB组织提取物的蛋白质免疫印迹和NB的石蜡切片中检测这些蛋白质。
蛋白质免疫印迹表明，NB肿瘤提取物含有与来自正常组织的大鼠外周蛋白和人NF-66/α-中间丝蛋白共迁移的免疫反应性蛋白，从而确定了这些抗体在肿瘤中对这些蛋白质的特异性。针对外周蛋白的特异性抗体标记了所有NB，包括未成熟NB、复合型神经节神经母细胞瘤和神经节瘤。相比之下，NF-66/α-中间丝蛋白抗体仅标记了50%的NB，并且仅微弱标记了大多数神经节神经母细胞瘤和神经节瘤。两种抗体均未标记其他小细胞蓝染肿瘤，如淋巴瘤、横纹肌肉瘤、肾母细胞瘤和尤因肉瘤。
外周蛋白对NB标记的特异性以及外周蛋白抗体标记包括神经节神经母细胞瘤和神经节瘤在内的整个NB谱的能力表明，这种中间丝蛋白有潜力作为这些相关神经嵴肿瘤的诊断标志物。
