L-pipecolaturia in Zellweger syndrome.
Purified rat peroxisomes have been reported to oxidize D-pipecolic acid and the pipecolaturia of Zellweger syndrome has been attributed to the absence of peroxisomes. The logical consequences would be excesses of D-pipecolic acid in the urine of patients with Zellweger syndrome. The urine of two patients with Zellweger syndrome has been analyzed by complexing the pipecolic acid to copper-aspartame to separate the L- and D-isomers. L-Pipecolic acid constituted 100% and 78% of the total pipecolic acid in the two urines. The possibility of preferential retention of D-pipecolic acid was excluded by measuring renal excretion in two control subjects following administration of each isomer. The clearance of L-pipecolic acid was 1.1 and 0.2 ml/min and of D-pipecolic acid was 36.4 and 43.6 ml/min. These results do not support the contention that the pipecolaturia of Zellweger syndrome is the direct result of peroxisomal deficiencies.
Lam
S
S
Hutzler
J
J
Dancis
J
J
D001927:Brain Diseases / Q000652:urine*; D003605:D-Amino-Acid Oxidase / Q000378:metabolism; D006801:Humans; D007674:Kidney Diseases / Q000652:urine*; D008107:Liver Diseases / Q000652:urine*; D010875:Pipecolic Acids / Q000652:urine*; D013237:Stereoisomerism; D013577:Syndrome
D010875:Pipecolic Acids; D003605:D-Amino-Acid Oxidase; C031345:pipecolic acid