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.
Wanders
R J
RJ
Department of Clinical Chemistry, University Hospital Amsterdam, The Netherlands.
Romeijn
G J
GJ
D019748:Carbon-Carbon Double Bond Isomerases; D002262:Carboxy-Lyases / Q000378:metabolism; D002784:Cholesterol / Q000096:biosynthesis*; D005347:Fibroblasts / Q000201:enzymology*; D045782:Hemiterpenes; D006801:Humans; D007535:Isomerases / Q000378:metabolism; D008099:Liver / Q000201:enzymology*; D017853:Phosphotransferases (Alcohol Group Acceptor) / Q000172:deficiency* / Q000378:metabolism; D015211:Zellweger Syndrome / Q000201:enzymology*
D045782:Hemiterpenes; D002784:Cholesterol; D017853:Phosphotransferases (Alcohol Group Acceptor); C021796:mevalonate kinase; D002262:Carboxy-Lyases; C022503:pyrophosphomevalonate decarboxylase; D007535:Isomerases; D019748:Carbon-Carbon Double Bond Isomerases; C021453:isopentenyldiphosphate delta-isomerase