L-pipecolaturia in Zellweger syndrome.

Abstract

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.

• Authors: S Lam, J Hutzler, J Dancis
• Journal: Biochimica et biophysica acta (Biochim Biophys Acta) Vol. 882 Issue 2 Pg. 254-7 (Jun 19 1986) ISSN: 0006-3002 [Print] Netherlands
• PMID: 2871866 (Publication Type: Journal Article, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Pipecolic Acids
• D-Amino-Acid Oxidase
• pipecolic acid

Topics

• Brain Diseases (urine)
• D-Amino-Acid Oxidase (metabolism)
• Humans
• Kidney Diseases (urine)
• Liver Diseases (urine)
• Pipecolic Acids (urine)
• Stereoisomerism
• Syndrome