Niemann-Pick type C1 (NPC) disease is an autosomal recessive neurodegenerative disorder. One feature of the mouse model of NPC1 is it’s infertility. We have made transgenic mice which express the Npc1 protein exclusively in fibrillary astrocytes, using the glial fibrillary acidic protein (GFAP) promoter. This selective expression ofNpc1 corrects sterility in GFAP-Npc1^E,Npc1 ^-/- mice. Counts of acidophils in the pituitary of GFAP-Npc1^E,Npcl ^-/- mice, as compared toNpcl ^-/- mice, and measurements of dopamine D2 receptor (DRD2) mRNA in the pituitary, suggest mechanisms for fertility enhancement. We conclude that the correction of sterility in GFAP-Npc1^E,Npc1 ^-/- mice is a result of restoring hypothalamic control of the pituitary.

中文翻译：

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Npc1 在神经胶质细胞中的表达纠正了 Npc1(-/-) 小鼠的不育。

Niemann-Pick C1 型 (NPC) 疾病是一种常染色体隐性神经退行性疾病。NPC1 小鼠模型的一个特点是它的不孕症。我们使用胶质纤维酸性蛋白 (GFAP) 启动子制作了仅在纤维状星形胶质细胞中表达 Npc1 蛋白的转基因小鼠。Npc1 的这种选择性表达纠正了 GFAP-Npc1 ^E、Npc1 ^-/-小鼠的不育。在GFAP-NPC1的垂体嗜酸的计数^ë，NPCL ^{- / -}小鼠相比，NPCL ^{- / -}小鼠，以及垂体中多巴胺 D2 受体 (DRD2) mRNA 的测量，提示了生育力增强的机制。我们得出结论，GFAP-Npc1 ^E、Npc1 ^-/-小鼠不育的纠正是恢复垂体下丘脑控制的结果。