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Tytuł artykułu

Expression of Npc1 in glial cells corrects sterility in Npc1 mice

Warianty tytułu

Języki publikacji

EN

Abstrakty

EN
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.

Wydawca

-

Rocznik

Tom

50

Numer

4

Opis fizyczny

p.385-390,fig.,ref.

Twórcy

autor
  • Department of Pediatrics, University of Arizona, 1501 N. Campbell Avenue, PO Box 245073, Tucson, AZ 85724-5073, USA
autor
  • Department of Physiology, University of Arizona, Tucson, Arizona, USA
autor
  • Department of Molecular and Cellular Biology, University of Arizona, Tucson, Arizona, USA

Bibliografia

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  • Erickson R, Kiela M, Devine P, Hoyer P, Heidenreich R, 2002. mdr1a deficiency corrects sterility in Niemann-Pick C1 protein deficient female mice. Mol Reprod Dev 62: 167-173.
  • Fan J, Akabane H, Graham S, Richardson L, Zhu G, 2006. Sperm defects in mice lacking a functional Niemann-Pick C1 protein. Mol Reprod Dev 73: 1284-1291.
  • Gafvels M, Bjurulf E, Selstam G, 1992. Prolactin stimulates the expression of luteinizing hormone/chorionic gonadotropin receptor messenger ribonucleic acid in the rat corpus luteum and rescues early pregnancy from bromocriptine-induced abortion. Biol Reprod 47: 534-540.
  • Garver WS, Heidenreich RH, 2002. The Niemann-Pick C proteins and trafficking of cholesterol through the late endosomal/lysosomal system. Curr Mol Med 2: 485-505.
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  • Patterson MC, Vanier MT, Suzuki K, Morris JA, Carstea ED, Neufeld EB, et al. 2001. Niemann-Pick disease type C: a lipid trafficking disorder. In: Scriver SR, Beaudet AL, Sly WS, Valle D, eds. The metabolic and molecular bases of inherited disease, 8th ed. New York: McGraw-Hill: 3611-3634.
  • Pentchev PG, Comly ME, Kruth HS, Vanier MT, Wenger DA, Patel S, Brady RO, 1985. A defect in cholesterol esterification in Niemann-Pick disease (type C) patients. Proc Natl Acad Sci USA 82: 8247-8251.
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  • Roff CF, Strauss JF 3rd, Goldin E, Jaffe H, Patterson MC, Agritellis GC, et al. 1993. The murine Niemann-Pick C lesion affects testosterone production. Endocrinology 133: 2913-2922.
  • Saiardi A, Bozzi Y, Ja-Hyum B, Borrelli E, 1997. Antiproliferative role of dopamine: loss of D₂ receptors causes hormonal dysfunction and pituitary hyperplasia. Neuron 19: 115-126.
  • Smith MS, Freeman ME, Neill MD, 1975. 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 pseudopregnancy. 96: 219-226.
  • Strauss JF 3rd, Liu K, Christenson LK, Watari H, 2002. Sterols and intracellular vesicular trafficking: lessons from the study of NPC1. Steroids 67: 947-951.
  • Xie C, Richardson JA, Turley SD, Dietschy JM, 2006. Cholesterol substrate pools and steroid hormone levels are normal in the face of mutational inactivation of NPC1 protein. J Lipid Res 47: 953-963.
  • Zhang M, Strnatka D, Donohue C, Hallows J, Vincent I, Erickson RP, 2008. Astrocyte-only Npcl reduces neuronal cholesterol and triples life span of Npcl-/- mice. J Neurosci Res 86: 2848-2856.

Typ dokumentu

Bibliografia

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