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Dhcr7  -  7-dehydrocholesterol reductase

Mus musculus

Synonyms: 7-DHC reductase, AA409147, Sterol Delta(7)-reductase
 
 
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Disease relevance of Dhcr7

 

High impact information on Dhcr7

 

Biological context of Dhcr7

 

Anatomical context of Dhcr7

  • Immunohistochemical analyses have revealed a 306% increase in the area of 5-HT immunoreactivity (5-HT IR) in the hindbrains of mutant (Dhcr7-/-) mice as compared to age-matched wild type animals [9].
 

Associations of Dhcr7 with chemical compounds

  • Because their heterozygous mothers synthesize cholesterol normally, virtually all sterols found in a Dhcr7 knockout fetus having a Delta(7) or a Delta(8) double bond must have been synthesized by the fetus itself but any cholesterol had to have come from the mother [6].
  • In addition, the rostral and caudal raphe groups exhibited a radial expansion in Dhcr7-/- mice, with 5-HT IR cells present in locations not seen in wild type mice [9].
 

Other interactions of Dhcr7

  • The suggested mechanism of hypocholesterolemic effect is the inhibition of squalene synthase and 7DHC reductase [10].
  • No significant differences of the spatial-temporal localization of sonic hedgehog (Shh) or its downstream targets by immunohistochemistry were detected between knockout and wild-type lungs and Shh autoprocessing occurred normally in tissues from Dhcr7-/- embryos [8].

References

  1. Cholesterol deficiency in a mouse model of Smith-Lemli-Opitz syndrome reveals increased mast cell responsiveness. Kovarova, M., Wassif, C.A., Odom, S., Liao, K., Porter, F.D., Rivera, J. J. Exp. Med. (2006) [Pubmed]
  2. Immunohistochemical and microarray analyses of a mouse model for the smith-lemli-opitz syndrome. Waage-Baudet, H., Dunty, W.C., Dehart, D.B., Hiller, S., Sulik, K.K. Dev. Neurosci. (2005) [Pubmed]
  3. 7-Dehydrocholesterol-dependent proteolysis of HMG-CoA reductase suppresses sterol biosynthesis in a mouse model of Smith-Lemli-Opitz/RSH syndrome. Fitzky, B.U., Moebius, F.F., Asaoka, H., Waage-Baudet, H., Xu, L., Xu, G., Maeda, N., Kluckman, K., Hiller, S., Yu, H., Batta, A.K., Shefer, S., Chen, T., Salen, G., Sulik, K., Simoni, R.D., Ness, G.C., Glossmann, H., Patel, S.B., Tint, G.S. J. Clin. Invest. (2001) [Pubmed]
  4. Development and characterization of a hypomorphic Smith-Lemli-Opitz syndrome mouse model and efficacy of simvastatin therapy. Correa-Cerro, L.S., Wassif, C.A., Kratz, L., Miller, G.F., Munasinghe, J.P., Grinberg, A., Fliesler, S.J., Porter, F.D. Hum. Mol. Genet. (2006) [Pubmed]
  5. Abnormal sterols in cholesterol-deficiency diseases cause secretory granule malformation and decreased membrane curvature. Gondré-Lewis, M.C., Petrache, H.I., Wassif, C.A., Harries, D., Parsegian, A., Porter, F.D., Loh, Y.P. J. Cell. Sci. (2006) [Pubmed]
  6. The use of the Dhcr7 knockout mouse to accurately determine the origin of fetal sterols. Tint, G.S., Yu, H., Shang, Q., Xu, G., Patel, S.B. J. Lipid Res. (2006) [Pubmed]
  7. Partial rescue of neonatal lethality of Dhcr7 null mice by a nestin promoter-driven DHCR7 transgene expression. Yu, H., Wessels, A., Tint, G.S., Patel, S.B. Brain Res. Dev. Brain Res. (2005) [Pubmed]
  8. Late gestational lung hypoplasia in a mouse model of the Smith-Lemli-Opitz syndrome. Yu, H., Wessels, A., Chen, J., Phelps, A.L., Oatis, J., Tint, G.S., Patel, S.B. BMC Dev. Biol. (2004) [Pubmed]
  9. Abnormal serotonergic development in a mouse model for the Smith-Lemli-Opitz syndrome: implications for autism. Waage-Baudet, H., Lauder, J.M., Dehart, D.B., Kluckman, K., Hiller, S., Tint, G.S., Sulik, K.K. Int. J. Dev. Neurosci. (2003) [Pubmed]
  10. RPR 101821, a new potent cholesterol-lowering agent: inhibition of squalene synthase and 7-dehydrocholesterol reductase. Amin, D., Rutledge, R.Z., Needle, S.J., Hele, D.J., Neuenswander, K., Bush, R.C., Bilder, G.E., Perrone, M.H. Naunyn Schmiedebergs Arch. Pharmacol. (1996) [Pubmed]
 
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