Arii N, Mizuguchi M, Mori K, Takashima S
Department of Mental Retardation and Birth Defect Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan.
Acta Neuropathol. 2000 Nov;100(5):506-12. doi: 10.1007/s004010000184.
Telencephalin (TLN), a telencephalon-specific glycoprotein, is exclusively expressed in neurons of the mammalian telencephalon. In the normally developing human brain, TLN immunoreactivity appeared and increased from 35 gestational weeks (GW) in the temporal cortex, and reached adult level at 5 months of postnatal age, being strong in the molecular layer, and weak in the external and internal granular layers. TLN expression corresponded with the development of neuronal dendrites and synapses. In brains with holoprosencephaly TLN immunoreactivity was already strong from as early as 28 GW. Staining was weak in the molecular layer, but strong in the external sparse and middle cellular layers in most cases. Notably, TLN was abundant in the glomerular structures in the internal pyramidal and multiform layers of fetal brains with alobar holoprosencephaly, which disappeared with increasing age. These results indicate premature and ectopic development of the dendrites and synaptic network in holoprosencephaly.
端脑糖蛋白（TLN）是一种端脑特异性糖蛋白，仅在哺乳动物端脑的神经元中表达。在正常发育的人类大脑中，TLN免疫反应性从妊娠35周（GW）开始在颞叶皮质出现并增加，并在出生后5个月达到成人水平，在分子层较强，在外部和内部颗粒层较弱。TLN表达与神经元树突和突触的发育相对应。在患全前脑畸形的大脑中，TLN免疫反应性早在28GW时就已经很强。在大多数情况下，分子层染色较弱，但在外部稀疏层和中间细胞层较强。值得注意的是，在无脑叶全前脑畸形胎儿大脑的内锥体层和多形层的肾小球结构中，TLN丰富，随着年龄增长而消失。这些结果表明全前脑畸形中树突和突触网络的过早和异位发育。
