Abstract
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-Npc1E,Npc1 -/- mice. Counts of acidophils in the pituitary of GFAP-Npc1E,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-Npc1E,Npc1 -/- mice is a result of restoring hypothalamic control of the pituitary.
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Donohue, C., Marion, S. & Erickson, R.P. Expression ofNpc1 in Glial Cells Corrects Sterility inNpc1 -/- mice. J Appl Genet 50, 385–390 (2009). https://doi.org/10.1007/BF03195698
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DOI: https://doi.org/10.1007/BF03195698