Reproducing abnormal cholesterol biosynthesis as seen in the Smith-Lemli-Opitz syndrome by inhibiting the conversion of 7-dehydrocholesterol to cholesterol in rats.

@article{Xu1995ReproducingAC,
  title={Reproducing abnormal cholesterol biosynthesis as seen in the Smith-Lemli-Opitz syndrome by inhibiting the conversion of 7-dehydrocholesterol to cholesterol in rats.},
  author={Guorong Xu and Gerald Salen and Sarah Shefer and Gene C. Ness and Theresa S. Chen and Zhihong Zhao and G. Stephen Tint},
  journal={The Journal of clinical investigation},
  year={1995},
  volume={95 1},
  pages={
          76-81
        }
}
The Smith-Lemli-Opitz syndrome is a recessive inherited disorder characterized by neurologic developmental defects and dysmorphic features in many organs. Recently, abnormal cholesterol biosynthesis with impaired conversion of 7-dehydrocholesterol to cholesterol has been discovered in homozygotes. To reproduce the biochemical abnormality, BM 15.766, a competitive inhibitor of 7-dehydrocholesterol-delta 7-reductase, the enzyme that catalyzes the conversion of 7-dehydrocholesterol into… CONTINUE READING

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RPR 101821, a new potent cholesterol-lowering agent: inhibition of squalene synthase and 7-dehydrocholesterol reductase

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  • 1996
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References

Publications referenced by this paper.
SHOWING 1-10 OF 19 REFERENCES

Differing effects of cholesterol and taurocholate on steady state hepatic HMG - CoA reductase and cholesterol 7 a - hydroxylase activities and mRNAlevels in the rat

G. Salen, G. S. Tint A. K. Batta, M. Irons
  • J . Lipid Res .
  • 1994

Methods of assay . In Cholesterol 7 a - hydroxylase ( 7 a - monooxygenase )

A. L. Farr, R. J. Randall
  • 1986

The effects of BM 15 . 766 , An inhibitor of 7 - dehydrocholesterol A ' - reductase , on cholesterol biosynthesis in primary rat hepatocytes

F. H. Schmidt, K. Stegmeier, E. C. Witte
  • Biochem . Pharmacol .
  • 1986