Identification of L-pipecolate oxidase in human liver and its deficiency in the Zellweger syndrome.
The ability of human liver to oxidize L-pipecolic acid was investigated. Liver from control subjects was found to contain L-pipecolic acid oxidase, an H2O2-producing enzyme not previously demonstrated in mammals. In livers from patients with the cerebro-hepato-renal syndrome of Zellweger, a genetic disease characterized by the absence of morphologically distinguishable peroxisomes, L-pipecolic acid oxidase was found to be deficient. These results indicate that L-pipecolic acid oxidase is a peroxisomal enzyme in man and provide an explanation for the fact that elevated levels of L-pipecolic acid are found in body fluids of patients with the Zellweger syndrome.
Wanders
R J
RJ
Department of Pediatrics, University Hospital Amsterdam, The Netherlands.
Romeyn
G J
GJ
van Roermund
C W
CW
Schutgens
R B
RB
van den Bosch
H
H
Tager
J M
JM
D000217:Acyltransferases / Q000378:metabolism; D002374:Catalase / Q000378:metabolism; D030342:Genetic Diseases, Inborn / Q000201:enzymology*; D006801:Humans; D008099:Liver / Q000201:enzymology*; D000587:Oxidoreductases Acting on CH-NH Group Donors / Q000378:metabolism*; D012016:Reference Values; D013577:Syndrome
D002374:Catalase; D000587:Oxidoreductases Acting on CH-NH Group Donors; C032771:L-pipecolate dehydrogenase; D000217:Acyltransferases; C020051:glycerone-phosphate O-acyltransferase