Normal and inhibited cholesterol synthesis in the cultured rat embryo

Journal of Lipid Research

Volumn 38, Issue 1, 1997, Pages 22-34

  Llirbat, Beatrice ^a   Wolf, Claude ^a   Chevy, Francoise ^a   Citadelle, Danielle ^b   Bereziat, Gilbert ^a   Roux, Charles ^b  

^a HÔPITAL SAINT ANTOINE   (France)

^b Section of Radiology   (France)

Author keywords

7 dehydrochlesterol reductase; 8 7 sterol isomerase; 13C labeled precursors; AY 9944; mass spectrometry; Smith Lemki Opitz syndrome

Indexed keywords

1,4 BIS(2 CHLOROBENZYLAMINOMETHYL)CYCLOHEXANE; 7 DEHYDROCHOLESTEROL; CHOLESTEROL; OXIDOREDUCTASE;

ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; CHOLESTEROL SYNTHESIS; CULTURE MEDIUM; DIET SUPPLEMENTATION; EMBRYO; EMBRYO CULTURE; EMBRYO DEVELOPMENT; FEMALE; FETUS MALFORMATION; HYPERCHOLESTEROLEMIA; HYPOCHOLESTEROLEMIA; LIPID TRANSPORT; MASS SPECTROMETRY; METABOLIC DISORDER; MOTHER FETUS RELATIONSHIP; NONHUMAN; PRIORITY JOURNAL; RAT; SMITH LEMLI OPITZ SYNDROME;

ADAPTATION, PHYSIOLOGICAL; ANIMALS; ANTICHOLESTEREMIC AGENTS; CHOLESTEROL; CULTURE TECHNIQUES; DRUG EVALUATION, PRECLINICAL; EMBRYO; ENZYME INHIBITORS; MOLECULAR STRUCTURE; OXIDOREDUCTASES; OXIDOREDUCTASES ACTING ON CH-CH GROUP DONORS; RATS; RATS, WISTAR; REFERENCE VALUES; TRANS-1,4-BIS(2-CHLOROBENZAMINOMETHYL)CYCLOHEXANE DIHYDROCHLORIDE;

EID: 0031027149     PISSN: 00222275     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (38)

1. Irons, M., E. R. Ellias, G. Salen, G. S. Tint, and A. K. Batta. 1993. Defective cholesterol biosynthesis in Smith-Lemli-Opitz syndrome. Lancet. 341:1414.
2. Tint, G. S., M. Irons, E. R. Elias, A. K. Batta, R. Frieden, T. S. Chen, and G. Salen. 1994. Defective cholesterol biosynthesis associated with the Smith-Lemli-Opitz syndrome. N. Engl. J. Med. 330: 107-113.
3. Tint, G. S., M. Seller, R. Hughes-Benzie, A. K. Batta, S. Shefer, D. Genest, M. Irons, E. Elias, and G. Salen. 1995. Markedly increased tissue concentrations of 7-dehydrocholesterol combined with low levels of cholesterol are characteristic of the Smith-Lemli-Opitz syndrome. J. Lipid Res. 36: 89-95.
4. Batta, A. K., G. S. Tint, S. Shefer, D. Abuelo, and G. Salen. 1995. Identification of 8-dehydrocholesterol (cholesta-5,8-dien-3β-ol) in patients with Smith-Lemli-Opitz syndrome. J. Lipid Res. 36: 705-712.
5. Batta, A. K., G. Salen, G. S. Tint, and S. Shefer. 1995. Identification of 19-nor-5.7,9(10)-cholestatrien-3β-ol in patients with Smith-Lemli-Opitz syndrome. J. Lipid Res. 36: 3413-2418.
6. 7 bond of 7-dehydrocholesterol. J. Biol. Chem. 237: 358-362.
7. 7-reductase activity in liver microsomes from Smith-Lemli-Opitz homozygotes. J. Clin. Invest. 96: 1779-1785.
8. Honda, A., G. S. Tint, G. Salen, A. K. Batta, T. S. Chen, and S. Shefer. 1995. Defective conversion of 7-dehydrocholesterol to cholesterol in cultured skin fibroblasts from Smith-Lemli-Opitz syndrome homozygotes. J. Lipid. Res. 36: 1595-1601.
9. Alley, T. L., B. A. Gray, S. H. Lee, S. W. Scherer, L. C. Tsui, G. S. Tint, C. A. Williams, R. Zori, and M. R. Wallace. 1995. Identification of a yeast artificial chromosome clone spanning a translocation breakpoint at 7q32.1 in a Smith-Lemli-Opitz syndrome patient. Am. J. Hum. Genet. 56: 1411-1416.
10. Roux, C., and M. Aubry. 1966. Action teratogene d'un inhibiteur de la synthese du cholesterol, le AY 9944. C. R. Soc. Biol. 160: 1353-1357.
11. Barbu, V., C. Roux, D. Lambert, R. Dupuis, J. Gardette, J. C. Maziere, C. Maziere, E. Elefant, and J. Polonovski. 1988. Cholesterol prevents the teratogenic action of AY 9944: importance of the timing of cholesterol supplementation to rats. J. Nutr. 118: 774-779.
12. 7-reductase inhibitors: comparison to levels in humans with Smith-Lemli-Opitz syndrome. J. Lipid Res. 37: 1325-1333.
13. Roux, C., R. Dupuis, C. Horvath, and A. Giroud. 1979. Interpretation of isolated agenesis of the pituitary. Teratology. 19: 39-44.
14. Roux, C., R. Dupuis, C. Horvath, and J. N. Talbot. 1980. Teratogenic effect of an inhibitor of cholesterol synthesis (AY 9944) in rats: correlation with maternal cholesterolemia. J. Nutr. 110: 2310-2312.
15. Barbu, V., C. Roux, R. Dupuis, J. Gardette, and J. C. Maziere. 1984. Teratogenic effect of AY 9944 in rats: importance of the day of administration and maternal plasma cholesterol level. Proc. Soc. Exp. Biol. Med. 176: 54-59.
16. Roux, C., C. Wolf, N. Mulliez, M. Kolf, D. Citadelle, F. Chevy, and M. O. Delaunay. 1995. Teratogenic action in rats of a new 7-dehydrocholesterol reductase inhibitor: BM 15766. European Teratology Soc. Abstr. Teratology. 51: 29A,36.
17. Kelley, R. I. 1995. Diagnosis of Smith-Lemli-Opitz syndrome by gas chromatography/mass spectrometry of 7-dehydrocholesterol in plasma, amniotic fluid and cultured skin fibroblasts. Clin. Chim. Acta. 236: 45-58.
18. Xu, G., G. Salen, S. Shefer, G. C. Ness, T. S. Chen, Z. Zhao, and G. S. Tint. 1995. Reproducing abnormal cholesterol biosynthesis as seen in the Smith-Lemli-Opitz syndrome by inhibiting the conversion of 7-dehydrocholesterol to cholesterol in rats. J. Clin. Invest. 95: 76-81.
19. Brown, N. A., and S. Fabro. 1981. Quantification of rat embryonic development in vitro: a morphological scoring system. Teratology. 24: 65-78.
20. Wolthers, B. G., H. T. Walrecht, J. C. van der Molen, G. T. Nagel, J. J. Van Doormal, and P. N. Wijnandts. 1991. Use of determinations of 7-lathosterol (5α-cholest-7-en3β-ol) and other cholesterol precursors in serum in the study and treatment of disturbances of sterol metabolism, particularly cerebrotendinous xanthomatosis. J. Lipid Res. 32: 603-612.
21. Millard, B. J. 1979. Quantitative Mass Spectrometry. Heyden, editor. London. 66.
22. 7-reductase in higher plant microsomes. Biochem. Biophys. Res. Commun. 181: 465-473.
23. Plotz, E. J., J. J. Kabara, M. E. Davis, G. V. Leroy, and R. Gordon Gould. 1968. Studies on the synthesis of cholesterol in the brain of the human foetus. Am. J. Obst. Gynecol. 101: 534-538.
24. Pratt, H. P. M.. 1982. Preimplantation mouse embryos synthesize membrane sterols. Dev. Biol. 89: 101-110.
25. Brewer, L. M., S. A. Sheardown, and N. A. Brown. 1993. HMG-CoA reductase mRNA in the post-implantation rat embryo studied by in situ hybridization. Teratology. 47: 137-146.
26. Chevallier, F. 1964. Transferts et synthese du cholesterol chez le rat au cours de sa croissance (Transfer and synthesis of cholesterol in rats during their growth). Biochim. Biophys. Acta. 84: 316-339.
27. Brown, N. A., D. O. Clarke, and A. McCarthy. 1991. Adaptation of postimplantation embryos to culture: membrane lipid synthesis and response to valproate. Reprod. Toxicol. 5: 245-253.
28. Polonovski, J., J. C. Maziere, C. Maziere, L. Mora, F. Gallie, J. Gardette, V. Barbu, and C. Roux. 1986. Effect of amphiphilic drugs on low density lipoprotein and sterol metabolism in cultured human fibroblasts. In Lipid Metabolism and Its Pathology. M. J. Halpern, editor. Elsevier Science Publishers B. V. 19-27.
29. Pratt Rossiter, J., K. J. Hofman, and . I. Kelley. 1995. Smith-Lemli-Opitz syndrome: prenatal diagnosis by quantification of cholesterol precursors in amniotic fluid. Am. J. Med. Genet. 56: 272-275.
30. Repetto, M., J. C. Maziere, D. Citadelle, R. Dupuis, M. Meier, S. Biade, D. Quiec, and C. Roux. 1990. Teratogenic effect of the cholesterol synthesis inhibitor AY 9944 on rat embryos in vitro. Teratology. 42: 611-618.
31. Roux, C., C. Horvath, and R. Dupuis. 1979. Teratogenic action and embryo lethality of AY 9944: prevention by a hypercholesterolemia-provoking diet. Teratology. 19: 35-38.
32. Farese, R. V., S. Cases, S. L. Ruland, H. J. Kayden, J. S. Wong, S. G. Young, and R. L. Hamilton. 1996. A novel function for apolipoprotein B: lipoprotein synthesis in the yolk sac is critical for maternal-fetal lipid transport in mice. J. Lipid Res. 37: 347-360.
33. Homanics, G. E., T. J. Smith, S. H. Zhang, D. Lee, S. G. Young, and N. Maeda. 1993. Targeted modification of the apolipoprotein B gene results in hypobetalipoproteinemia and developmental abnormalities in mice. Proc. Natl. Acad. Sci. USA. 90: 2389-2393.
34. Lanoue, L., D. B. Dehart, S. Tint, N. Maeda, and K. K. Sulik. 1996. Gene-environment interactions in cholesterol deficiency-based malformations. Teratology Soc. Abstr. Teratology. 53: A26, 89.
35. Lin, D. S., R. M. Pitkin, and W. E. Connor. 1977. Placental transfer of cholesterol into the human fetus. Am. J. Obstet. Gynecol. 128: 735-739.
36. Pardridge, W. M., and L. J. Mietus. 1980. Palmitate and cholesterol transport through the blood-brain barrier. J. Neurochem. 34: 463-466.
37. Serouge, C., C. Lefevre, and F. Chevallier. 1976. Cholesterol transfer between brain and plasma in the rat: a model for the turnover of cerebral cholesterol. Exp. Neurol. 51: 229-240.
38. Axelson, M. 1991. Occurrence of isomeric dehydrocholesterols in human plasma. J. Lipid Res. 32: 1441-1448.