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Expression of Npc1 in glial cells corrects sterility in Npc1 mice

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

Journal of Applied Genetics

2009

tom 50

nr 4

385-390

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 Npcl protein exclusively in fibrillary astrocytes, using the glial fibrillary acidic protein (GFAP) promoter. This selective expression of Npcl corrects sterility in GFAP-Npc1E, Npcl-/- 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.

Ograniczanie wyników

1 Journal of Applied Genetics

1 Donohue C.

1 Erickson R. P.

1 Marion S.

1 2009

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Expression of Npc1 in glial cells corrects sterility in Npc1 mice