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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)
 
Chemical Compound Review

AC1NSU9V     (3S,10R,13R,17R)-10,13- dimethyl-17-(6...

Synonyms:
 
 
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Disease relevance of 7-Dehydrocholesterol

 

High impact information on 7-Dehydrocholesterol

  • The Smith-Lemli-Opitz syndrome (SLOS) is caused by deficient Delta(7)-dehydrocholesterol reductase, which catalyzes the final step of the cholesterol biosynthetic pathway, resulting in low cholesterol and high concentrations of its direct precursors 7-dehydrocholesterol (7DHC) and 8DHC [2].
  • The offspring phenotype remained deleterious whether or not the dams received 7DHC-rich diet [1].
  • We hypothesized that i) 7DHC and 8DHC accumulation contributes to the poor outcome of SLOS patients and ii) blood exchange transfusions with hydroxymethylglutaryl (HMG)-CoA reductase inhibition would improve the precursor-to-cholesterol ratio and may improve the clinical outcome of SLO patients [2].
  • Cholesterol concentrations were abnormally low in cord blood and in the baby's plasma at 12 weeks, while levels of 7DHC were grossly elevated, confirming the prenatal diagnosis [3].
  • These data suggest that the defect in ecd1 tissue-mediated ecdysteroidogenesis does not involve a "hit" on any of the enzymes involved in either the 7,8-dehydrogenation of C or 25C or in the subsequent oxidation of 7d25C or 7dC to ecdysteroid [4].
 

Anatomical context of 7-Dehydrocholesterol

 

Associations of 7-Dehydrocholesterol with other chemical compounds

 

Analytical, diagnostic and therapeutic context of 7-Dehydrocholesterol

  • In dog and cat skin the concentrations of nonesterified 7DHC were below the detection limit of the HPLC system [5].

References

  1. Cholesterol deficit but not accumulation of aberrant sterols is the major cause of the teratogenic activity in the Smith-Lemli-Opitz syndrome animal model. Gaoua, W., Wolf, C., Chevy, F., Ilien, F., Roux, C. J. Lipid Res. (2000) [Pubmed]
  2. Simvastatin. A new therapeutic approach for Smith-Lemli-Opitz syndrome. Jira, P.E., Wevers, R.A., de Jong, J., Rubio-Gozalbo, E., Janssen-Zijlstra, F.S., van Heyst, A.F., Sengers, R.C., Smeitink, J.A. J. Lipid Res. (2000) [Pubmed]
  3. Prenatal detection of the cholesterol biosynthetic defect in the Smith-Lemli-Opitz syndrome by the analysis of amniotic fluid sterols. Abuelo, D.N., Tint, G.S., Kelley, R., Batta, A.K., Shefer, S., Salen, G. Am. J. Med. Genet. (1995) [Pubmed]
  4. Differential incorporation of cholesterol and cholesterol derivatives into ecdysteroids by the larval ring glands and adult ovaries of Drosophila melanogaster: a putative explanation for the l(3)ecd1 mutation. Warren, J.T., Bachmann, J.S., Dai, J.D., Gilbert, L.I. Insect Biochem. Mol. Biol. (1996) [Pubmed]
  5. Dietary vitamin D dependence of cat and dog due to inadequate cutaneous synthesis of vitamin D. How, K.L., Hazewinkel, H.A., Mol, J.A. Gen. Comp. Endocrinol. (1994) [Pubmed]
 
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