Expression ofNpc1 in Glial Cells Corrects Sterility inNpc1 -/- mice
- 1 December 2009
- journal article
- Published by Springer Nature in Journal of Applied Genetics
- Vol. 50 (4) , 385-390
- https://doi.org/10.1007/bf03195698
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.Keywords
This publication has 21 references indexed in Scilit:
- Astrocyte‐only Npc1 reduces neuronal cholesterol and triples life span of Npc1–/– miceJournal of Neuroscience Research, 2008
- Sperm defects in mice lacking a functional Niemann–Pick C1 proteinMolecular Reproduction and Development, 2006
- Cholesterol substrate pools and steroid hormone levels are normal in the face of mutational inactivation of NPC1 proteinJournal of Lipid Research, 2006
- Transgenic mouse models of dopamine deficiencyAnnals of Neurology, 2003
- Analysis of Relative Gene Expression Data Using Real-Time Quantitative PCR and the 2−ΔΔCT MethodMethods, 2001
- The murine Niemann-Pick type C lesion affects testosterone production.Endocrinology, 1993
- Prolactin Stimulates the Expression of Luteinizing Hormone/Chorionic Gonadotropin Receptor Messenger Ribonucleic Acid in the Rat Corpus Luteum and Rescues Early Pregnancy from Bromocriptin-Induced Abortion1Biology of Reproduction, 1992
- A defect in cholesterol esterification in Niemann-Pick disease (type C) patients.Proceedings of the National Academy of Sciences, 1985
- The Role of Lipoproteins in Steroidogenesis and Cholesterol Metabolism in Steroidogenic Glands*Endocrine Reviews, 1982
- The Control of Progesterone Secretion During the Estrous Cycle and Early Pseudopregnancy in the Rat: Prolactin, Gonadotropin and Steroid Levels Associated with Rescue of the Corpus Luteum of Pseudopregnancy12Endocrinology, 1975