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结节性硬化症胎儿脑部的免疫细胞化学研究。

Immunocytochemical studies on a fetal brain of tuberous sclerosis.

作者信息

Nishimura M, Takashima S, Takeshita K, Tanaka J

机构信息

Division of Child Neurology, Tottori University School of Medicine, Yonago, Japan.

出版信息

Pediatr Neurol. 1985 Jul-Aug;1(4):245-8. doi: 10.1016/s0887-8994(85)80010-2.

Abstract

A female stillborn baby was delivered at 36 weeks' gestation. There were rhabdomyomas in the heart, multi-cystic kidneys, and tubers in the brain. Neuropathologic examination of the brain tissue revealed typical giant cells in the sub-ependymal nodules, cerebral white matter, and cortex. On immunocytochemical staining, the giant cells stained positively with antisera to glial fibrillary acidic protein (GFAP), myelin basic protein (MBP), and neuron-specific enolase (NSE). The giant cells as well as adjacent glial cells with small eccentric nuclei contained both MBP and GFAP. Both cell types combined immunocytochemical characteristics of astrocytes and oligodendrocytes. There were also intermediate-sized cells found among giant cells and germ cells in subependymal nodules. In white matter nodules, similar intermediate-sized cells were found among giant cells and glial cells with small eccentric nuclei. Therefore, giant cells as well as glial cells with small eccentric nuclei may be multi-potential and may result from abnormal patterns of maturation.

摘要

一名妊娠36周的女性死产婴儿出生。其心脏有横纹肌瘤、多囊肾,脑部有结节。对脑组织进行神经病理学检查发现,室管膜下结节、脑白质和皮质中有典型的巨细胞。免疫细胞化学染色显示,巨细胞对胶质纤维酸性蛋白(GFAP)、髓鞘碱性蛋白(MBP)和神经元特异性烯醇化酶(NSE)的抗血清呈阳性染色。巨细胞以及相邻的核小且偏位的胶质细胞同时含有MBP和GFAP。这两种细胞类型兼具星形胶质细胞和少突胶质细胞的联合免疫细胞化学特征。在室管膜下结节的巨细胞和生殖细胞中还发现了中等大小的细胞。在白质结节中,巨细胞和核小且偏位的胶质细胞中也发现了类似的中等大小的细胞。因此,巨细胞以及核小且偏位的胶质细胞可能具有多能性,可能是成熟模式异常所致。

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