Regulation of the last two enzymatic reactions in cholesterol biosynthesis in rats: Effects of BM 15.766, cholesterol, cholic acid, lovastatin, and their combinations

Hepatology

Volumn 24, Issue 2, 1996, Pages 435-439

  Honda, Akira ^a,b   Shefer, Sarah ^a   Salen, Gerald ^a,b   Xu, Guorong ^a,b   Batta, Ashok K ^a   Tint, G Stephen ^a,b   Honda, Megumi ^a,b   Chen, Tai C ^c   Holick, Michael F ^c  

^a RUTGERS NEW JERSEY MEDICAL SCHOOL   (United States)

^b VETERANS AFFAIRS MEDICAL CENTER   (United States)

^c BOSTON UNIVERSITY MEDICAL CENTER   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

4 [2 [1 (4 CHLOROCINNAMYL) 4 PIPERAZINYL]ETHYL]BENZOIC ACID; 7 DEHYDROCHOLESTEROL; CHOLESTEROL; CHOLIC ACID; COLESTYRAMINE; HYDROXYMETHYLGLUTARYL COENZYME A REDUCTASE; LATHOSTEROL; LIVER ENZYME; MEVINOLIN; OXIDOREDUCTASE; STEROL;

ANIMAL EXPERIMENT; ANIMAL TISSUE; ARTICLE; CHOLESTEROL SYNTHESIS; CONTROLLED STUDY; ENZYME ACTIVITY; MALE; NONHUMAN; ORAL DRUG ADMINISTRATION; PRIORITY JOURNAL; RAT;

EID: 0029783409     PISSN: 02709139     EISSN: None     Source Type: Journal    
DOI: 10.1053/jhep.1996.v24.pm0008690416     Document Type: Article

References (26)

1. Smith DW, Lemli L, Opitz JM. A newly recognized syndrome of multiple congenital anomalies. J Pediatr 1964;64:210-217.
2. Honda A, Tint GS, Salen G, Batta AK, Chen TS, Shefer S. Defective conversion of 7-dehydrocholesterol to cholesterol in cultured skin fibroblasts from Smith-Lemli-Opitz syndrome homozygotes. J Lipid Res 1995;36:1595-1601.
3. 7-reductase activity in liver microsomes from Smith-Lemli-Opitz homozygotes. J Clin Invest 1995;96: 1779-1785.
4. Irons M, Elias ER, Salen G, Tint GS, Batta AK. Defective cholesterol biosynthesis in Smith-Lemli-Opitz syndrome. Lancet 1993;341:1414.
5. Tint GS, Irons M, Elias ER, Batta AK, Frieden R, Chen TS, Salen G. Defective cholesterol biosynthesis associated with the Smith-Lemli-Opitz syndrome. N Engl J Med 1994;330:107-113.
6. Chasalow FI, Blethen SL, Taysi IC Possible abnormalities of steroid secretion in children with Smith-Lemli-Opitz syndrome and their parents. Steroids 1985;46:827-843.
7. Curry CJR, Carey JC, Holland JS, Chopra D, Fineman R, Golabi M, Sherman S, et al. Smith-Lemli-Opitz syndrome-type II: multiple congenital anomalies with male pseudohermaphroditism and frequent early lethality. Am J Med Genet 1987;26:45-57.
8. Gorlin RJ, Cohen MM Jr., Levin LS. Syndromes of the head and neck. 3rd ed. New York, NY: Oxford University Press, 1990:890-895.
9. Pober B, Curry CJR, Rutledge JC. Smith-Lemli-Opitz syndrome. In: Buyse ML, ed. Birth defects encyclopedia. Dover, MA: Center for Birth Defects Information Services, in association with Blackwell Scientific; 1990:1570-1572.
10. Xu G, Salen G, Shefer S, Ness GC, Chen TS, Zhao Z, Tint GS. 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 1995;95:76-81.
11. Pill J, Schmidt FH, Stegmeier K, Witte E-C. Effects of BM 15.766 on serum lipids in rats. Horm Metabol Res 1985;17:543-544.
12. 7-reductase, on cholesterol biosynthesis in primary rat hepatocytes. Biochem Pharmacol 1986;35:911-916.
13. Xu G, Salen G, Shefer S, Ness GC, Chen TS, Zhao Z, Salen L, et al. Treatment of the cholesterol biosynthetic defect in Smith-Lemli-Opitz syndrome reproduced in rats by BM 15.766. Gastroenterology 1995;109:1301-1307.
14. Kawata S, Trzaskos JM, Gaylor JL. Microsomal enzymes of cholesterol biosynthesis from lanosterol. J Biol Chem 1985;260:6609-6617.
15. 7 bond of 7-dehydrocholesterol. J Biol Chem 1962;237:358-362.
16. Alberts AW, Chen J, Kuron G, Hunt V, Huff J, Hoffman C, Rothrock J, et al. Mevinolin: a highly potent competitive inhibitor of hydroxymethyl-glutaryl-coenzyme A reductase and cholesterol-lowering agent. Proc Natl Acad Sci U S A 1980;77:3957-3961.
17. Tint GS, Seller M, Hughes-Benzie R, Batta AK, Shefer S, Genest D, Irons M, et al. Markedly increased tissue concentrations of 7-dehydrocholesterol combined with low levels of cholesterol are characteristic of the Smith-Lemli-Opitz syndrome. J Lipid Res 1995;36:89-95.
18. Lowry OH, Rosebrough NJ, Farr AL, Randall RJ. Protein measurement with the Folin phenol reagent. J Biol Chem 1951;193:265-275.
19. Shefer S, Hauser S, Lapar V, Mosbach EH. Regulatory effects of sterols and bile acids on hepatic 3-hydroxy-3-methylglutaryl CoA reductase and cholesterol 7α-hydroxylase in the rat. J Lipid Res 1973;14:573-580.
20. Bilheimer DW, Grundy SM, Brown MS, Goldstein JL. Mevinolin and colestipol stimulate receptor-mediated clearance of low-density lipoprotein from plasma in familial hypercholesterolemia heterozygotes. Proc Natl Acad Sci U S A 1983;80:4124-4128.
21. Shefer S, Nguyen LB, Salen G, Ness GC, Chowdhary IR, Lerner S, Batta AK, et al. Differing effects of cholesterol and taurocholate on steady state hepatic HMG-CoA reductase and cholesterol 7α-hydroxylase activities and messenger RNA (mRNA) levels in the rat. J Lipid Res 1992;33:1193-1200.
22. Rodwell VW, Nordstrom JL, Mitschelen JJ. Regulation of HMG-CoA reductase. Adv Lipid Res 1976;14:1-69.
23. Björkhem I. Effects of mevinolin in rat liver: evidence for a lack of coupling between synthesis of hydroxymethylglutaryl-CoA reductase and cholesterol 7α-hydroxylase activity. Biochim Biophys Acta 1986;877:43-49.
24. Faust JR, Goldstein JL, Brown MS. Synthesis of ubiquinone and cholesterol in human fibroblasts: regulation of branched pathway. Arch Biochem Biophys 1979;192:86-99.
25. Faust JR, Goldstein JL, Brown MS. Squalene synthetase activity in human fibroblasts: regulation via the low density lipoprotein receptor. Proc Natl Acad Sci U S A 1979;76:5018-5022.
26. Hoffmann G, Gibson KM, Brandt IK, Bader PI, Wappner RS, Sweetman L. Mevalonic aciduria; an inborn error of cholesterol and nonsterol isoprene biosynthesis. N Engl J Med 1986;314:1610-1614.