L-pipecolaturia in Zellweger syndrome.
L-pipecolaturia in Zellweger syndrome.
复制标题
齐薇格综合征中的 L-管道尿尿。
DOI：
10.1016/0304-4165(86)90162-5
复制
发表时间：
1986
期刊：
影响因子：
--
通讯作者：
Dancis,J
中科院分区：
文献类型：
--
作者：
Lam,S;Hutzler,J;Dancis,J
Purified rat peroxisomes have been reported to oxidizeD-pipecolic acid and the pipecolaturia of Zellweger syndrome has been attributed to the absence of peroxisomes. The logical consequences would be excesses ofD-pipecolic acid in the urine of patients and Zellweger syndrome. The urine of two patients with Zellweger syndrome has been analyzed by complexing the pipecolic acid to copper-aspartame to separate theL- andD-isomers.L-Pipecolic acid constituted 100% and 78% of the total pipecolic acid in the two urines. The possibility of preferential retention ofD-pipecolic acid was excluded by measuring renal excretion in two control subjects following administration of each isomer. The clearance ofL-pipecolic acid was 1.1 and 0.2 ml/min and ofD-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.
