Bile acid biosynthesis in Smith-Lemli-Opitz syndrome bypassing cholesterol: Potential importance of pathway intermediates

Bile acids are the end products of cholesterol metabolism secreted into bile. They are essential for the absorption of lipids and lipid soluble compounds from the intestine. Here we have identified a series of unusual Δ(5)-unsaturated bile acids in plasma and urine of patients with Smith-Lemli-Opitz...

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Publicado en:J Steroid Biochem Mol Biol
Autores principales: Abdel-Khalik, Jonas, Hearn, Thomas, Dickson, Alison L., Crick, Peter J., Yutuc, Eylan, Austin-Muttitt, Karl, Bigger, Brian W., Morris, Andrew A., Shackleton, Cedric H., Clayton, Peter T., Iida, Takashi, Sircar, Ria, Rohatgi, Rajat, Marschall, Hanns-Ulrich, Sjövall, Jan, Björkhem, Ingemar, Mullins, Jonathan G.L., Griffiths, William J., Wang, Yuqin
Formato: Artigo
Lenguaje:Inglês
Publicado: Pergamon 2021
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Article
Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC7816163/
https://ncbi.nlm.nih.gov/pubmed/33246156
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.jsbmb.2020.105794
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