Literature DB >> 19875890

Expression of Npc1 in glial cells corrects sterility in Npc1(-/-) mice.

C Donohue1, 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-Npc1(-/-), Npc1(-/-) mice. Counts of acidophils in the pituitary of GFAP-Npc1E, Npc1(-/-) mice, as compared 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.

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Year:  2009        PMID： 19875890     DOI： 10.1007/BF03195698

Source DB:  PubMed          Journal:  J Appl Genet        ISSN： 1234-1983            Impact factor:   3.240


  20 in total

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Journal:  Endocrinology       Date:  1975-01       Impact factor: 4.736

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5.  mdr1a deficiency corrects sterility in Niemann-Pick C1 protein deficient female mice.

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Journal:  Mol Reprod Dev       Date:  2002-06       Impact factor: 2.609

6.  Aberrant intracellular cholesterol transport disrupts pituitary and ovarian function.

Authors:  Nicolas Y Gévry; Flavia L Lopes; Sandra Ledoux; Bruce D Murphy
Journal:  Mol Endocrinol       Date:  2004-04-22

Review 7.  Transgenic mouse models of dopamine deficiency.

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8.  Astrocyte-only Npc1 reduces neuronal cholesterol and triples life span of Npc1-/- mice.

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9.  The murine Niemann-Pick type C lesion affects testosterone production.

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10.  Real-time PCR based on SYBR-Green I fluorescence: an alternative to the TaqMan assay for a relative quantification of gene rearrangements, gene amplifications and micro gene deletions.

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Review 2.  Current controversies in Niemann-Pick C1 disease: steroids or gangliosides; neurons or neurons and glia.

Authors:  Robert P Erickson
Journal:  J Appl Genet       Date:  2013-01-05       Impact factor: 3.240

Review 3.  The Extending Spectrum of NPC1-Related Human Disorders: From Niemann-Pick C1 Disease to Obesity.

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Review 4.  Do GWAS and studies of heterozygotes for NPC1 and/or NPC2 explain why NPC disease cases are so rare?

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Journal:  J Appl Genet       Date:  2018-09-13       Impact factor: 3.240

5.  Anatomically defined neuron-based rescue of neurodegenerative Niemann-Pick type C disorder.

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Journal:  J Neurosci       Date:  2011-03-23       Impact factor: 6.167

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  6 in total

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