Changes in serum sterols of rats treated with 7-dehydrocholesterol- Δ7-reductase inhibitors: Comparison to levels in humans with Smith-Lemli- Opitz syndrome

Journal of Lipid Research

Volumn 37, Issue 6, 1996, Pages 1325-1333

  Wolf, Claude ^a   Chevy, Françoise ^a   Pham, Jacques ^a   Kolf Clauw, Martine ^a   Citadelle, Danièle ^a   Mulliez, Nicole ^a   Roux, Charles ^a  

^a HÔPITAL SAINT ANTOINE   (France)

Author keywords

aberrant sterols profiling; animal disease model; cholesterol synthesis; Smith Lemli Opitz syndrome

Indexed keywords

1,4 BIS(2 CHLOROBENZYLAMINOMETHYL)CYCLOHEXANE; 4 [2 [1 (4 CHLOROCINNAMYL) 4 PIPERAZINYL]ETHYL]BENZOIC ACID; 7 DEHYDROCHOLESTEROL; OXIDOREDUCTASE; OXIDOREDUCTASE INHIBITOR; STEROL; STEROL DERIVATIVE;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CHILD; CHOLESTEROL BLOOD LEVEL; CHOLESTEROL INTAKE; CHOLESTEROL SYNTHESIS; CLINICAL ARTICLE; CONTROLLED STUDY; DIET SUPPLEMENTATION; DOSE RESPONSE; DRUG TISSUE LEVEL; ENZYME DEFECT; ENZYME INHIBITION; FEEDING; FETUS; HUMAN; INTOXICATION; LIPID ANALYSIS; NONHUMAN; PRIORITY JOURNAL; RAT; SMITH LEMLI OPITZ SYNDROME; TERATOGENICITY;

ANIMALS; CHILD; CHOLESTADIENOLS; CHOLESTEROL; DEHYDROCHOLESTEROLS; DISEASE MODELS, ANIMAL; ENZYME INHIBITORS; FEMALE; HETEROZYGOTE; HUMANS; OXIDOREDUCTASES; OXIDOREDUCTASES ACTING ON CH-CH GROUP DONORS; PIPERAZINES; PREGNANCY; PRENATAL EXPOSURE DELAYED EFFECTS; RATS; RATS, WISTAR; SMITH-LEMLI-OPITZ SYNDROME; STEROLS; TRANS-1,4-BIS(2-CHLOROBENZAMINOMETHYL)CYCLOHEXANE DIHYDROCHLORIDE;

EID: 0029891851     PISSN: 00222275     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (24)

1. 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.
2. 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.
3. Smith, D. W., L. Lemli, and J. M. Opitz. 1964. A newly recognized syndrome of multiple congenital abnomalies. J. Pediatr. 64: 210-217.
4. Batta, A. K., 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: 705-713.
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: 2413-2418.
6. 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.
7. 7-reductase activity in Smith-Lemli-Opitz syndrome. Am. J. Med. Genet. 50: 336.
8. Roux, C., and M. Aubry. 1996. Action tératogène d'un inhibiteur de la synthèse du cholesterol, le AY 9944. C. R. Soc. Biol. 160: 1353-1357.
9. Roux, C., C. Horvath, and R. Dupuis. 1979. Teratogenic action and embryolethality of AY 9944: prevention by hypercholesterolemia-provoking diet. Teratology. 19: 35-38.
10. Roux, C., C. Wolf, N. Mulliez, M. Kolf, D. Citadelle, F. Chevy, and M. D. Delaunay. 1995. Teratogenic action in rats of a new 7-dehydrocholesterol reductase inhibitor: BM 15766. European Teratology Soc. 23th Ann. Conf. (Dublin, Ireland) 4-7 Sept. 1995.
11. Barbu, V., C. Roux, R. Dupuis, J. Gardette, and J. C. Mazière. 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.
12. 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.
13. 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.
14. Barbu, V., C. Roux, D. Lambert, R. Dupuis, J. Gardette, J. C. Mazière, C. Mazière, 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.
15. Holick, M. F., N. M. Richtand, S. C. McNeill, M. Holick, J. E. Frommer, J. W. Henley, and J. T. Potts. 1979. Isolation and identification of previtamin D3 from the skin of rats exposed to ultraviolet irradiation. Biochemistry. 18: 1003-1009.
16. Axelson, M. 1991. Occurrence of isomeric dehydrocholesterols in human plasma. J. Lipid Res. 32:1441-1448.
17. Fieser, L. F., and G. Ourisson. 1953. Cholesterol and companions. IV. J. Am. Chem. Soc. 75: 4404-4414.
18. Batta, A. K., G. S. Tint, G. Salen, S. Shefer, M. Irons, and E. R. Elias. 1994. Identification of 7-dehydrocholesterol and related sterols in patients with Smith-Lemli-Opitz syndrome. Am. J. Med. Genet. 50: 334.
19. 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: 45-48.
20. Roux, C., M. Aubry, R. Dupuis, and C. Horvath. 1973. Effects tératogènes comparés du triparanol chez le rat Wistar et chez le rat Sprague-Dawley. C. R. Soc. Biol. 167: 1523-1526.
21. Roux. 1990. Teratogenic effect of the cholesterol synthesis inhibitor AY 9944 on rat embryos in vitro. Teratology. 42: 611-618.
22. Minsker, D. H., J. S. Macdonald, R. T. Robertson, and D. L. Bokelman. 1983. Mevalonate supplementation in pregnant rats suppresses the teratogenicity of mevinolinic acid, an inhibitor of 3-hydroxy-3-methylglutaryl-coenzyme A reductase. Teratology. 28: 449-456.
23. Surani, M. A. H., S. J. Kimber, and J. C. Osborn. 1983. Mevalonate reverses the developmental arrest of preimplantation mouse embryos by Compactin, an inhibitor of HMG-CoA reductase. J. Embryol. Exp. Morphol. 75: 205-223.
24. Natowicz, M. R., and J. E. Evans. 1994. Abnormal bile acids in the Smith-Lemli-Opitz syndrome. Am. J. Med. Genet. 50: 364-367.