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Journal of Applied Genetics 50(4), 2009, pp. 385-390

Expression of Npc1 in Glial Cells Corrects Sterility in Npc1-/- mice

C. Donohue, S. Marion, R. P. Erickson

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 of Npc1 corrects sterility in GFAP-Npc1E, Npc1-/- mice. Counts of acidophils in the pituitary of GFAP-Npc1E, Npc1-/- mice, as compared to Npc1-/- 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.

Key words: hypothalamus, neuronal-glial interactions, pituitary, steroid synthesis

Correspondence: R. P. Erickson, Department of Pediatrics, University of Arizona, 1501 N. Campbell Avenue, PO Box 245073, Tucson, AZ 85724-5073, USA; e-mail:

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