Phytanic acid oxidation in man: identification of a new enzyme catalysing the formation of 2-ketophytanic acid from 2-hydroxyphytanic acid and its deficiency in the Zellweger syndrome.
Wanders
R J
RJ
Academic Medical Centre, University of Amsterdam, Department of Pediatrics, The Netherlands.
van Roermund
C W
CW
Schor
D S
DS
ten Brink
H J
HJ
Jakobs
C
C
D006801:Humans; D007651:Keto Acids / Q000378:metabolism*; D008099:Liver / Q000201:enzymology; D008830:Microbodies / Q000201:enzymology; D010084:Oxidation-Reduction; D010088:Oxidoreductases / Q000302:isolation & purification; D010831:Phytanic Acid / Q000031:analogs & derivatives* / Q000378:metabolism*; D015211:Zellweger Syndrome / Q000201:enzymology*
D007651:Keto Acids; D010831:Phytanic Acid; C002585:2-hydroxyphytanic acid; D010088:Oxidoreductases