正常、弗里德赖希共济失调及二羧酸氨基酸尿症成纤维细胞中二羧酸氨基酸摄取情况

Dicarboxylic amino acid uptake in normal, Friedreich's ataxia, and dicarboxylic aminoaciduria fibroblasts.

作者信息

Melancon S B, Grenier B, Dallaire L, Potier M, Fontaine G, Grignon B, Geoffroy G, Lemieux B, Barbeau A

出版信息

Can J Neurol Sci. 1979 May;6(2):263-73. doi: 10.1017/s0317167100119766.

DOI:10.1017/s0317167100119766

Abstract

Glutamic and aspartic acid uptake was measured in skin fibroblasts from patients with Friedreich's Ataxia, dicarboxylic aminoaciduria, and normal individuals. The results showed no difference in uptake kinetics of either dicarboxylic amino acids between Friedreich's Ataxia and normal cells, but reduced uptake velocities in dicarboxylic aminoaciduria fibroblasts. Friedreich's Ataxia fibroblasts were, however, less calcium-dependent and more magnesium and phosphate-dependent than controls in glucose-free incubation mixture. This difference might be related to some degree of glucose intolerance by Friedreich's Ataxia fibroblasts in culture.

摘要

在患有弗里德赖希共济失调、二羧酸氨基酸尿症的患者以及正常个体的皮肤成纤维细胞中测量了谷氨酸和天冬氨酸的摄取。结果显示，弗里德赖希共济失调患者的细胞与正常细胞之间，二羧酸氨基酸的摄取动力学没有差异，但二羧酸氨基酸尿症成纤维细胞的摄取速度降低。然而，在无葡萄糖的孵育混合物中，弗里德赖希共济失调成纤维细胞比对照细胞对钙的依赖性更小，而对镁和磷酸盐的依赖性更大。这种差异可能在一定程度上与培养中的弗里德赖希共济失调成纤维细胞的某种程度的葡萄糖不耐受有关。