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Cholesterol biosynthesis in Zellweger syndrome: normal activity of mevalonate kinase, mevalonate-5'-pyrophosphate decarboxylase and IPP-isomerase in patients' fibroblasts but deficient mevalonate kinase activity in liver.

AuthorsR J Wanders, G J Romeijn
JournalJournal of inherited metabolic disease (J Inherit Metab Dis) Vol. 19 Issue 2 Pg. 193-6 ( 1996) ISSN: 0141-8955 [Print] United States
PMID8739963 (Publication Type: Journal Article)
Chemical References
  • Hemiterpenes
  • Cholesterol
  • Phosphotransferases (Alcohol Group Acceptor)
  • mevalonate kinase
  • Carboxy-Lyases
  • pyrophosphomevalonate decarboxylase
  • Isomerases
  • Carbon-Carbon Double Bond Isomerases
  • isopentenyldiphosphate delta-isomerase
Topics
  • Carbon-Carbon Double Bond Isomerases
  • Carboxy-Lyases (metabolism)
  • Cholesterol (biosynthesis)
  • Fibroblasts (enzymology)
  • Hemiterpenes
  • Humans
  • Isomerases (metabolism)
  • Liver (enzymology)
  • Phosphotransferases (Alcohol Group Acceptor) (deficiency, metabolism)
  • Zellweger Syndrome (enzymology)

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