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人二倍体成纤维细胞对甘氨酸的转运——非酮症高甘氨酸血症患者细胞无转运缺陷

Glycine transport by human diploid fibroblasts--absence of a defect in cells from patients with nonketotic hyperglycinemia.

作者信息

Kelly J C, Otto E F, Hillman R E

出版信息

Pediatr Res. 1979 Feb;13(2):127-30. doi: 10.1203/00006450-197902000-00008.

DOI:10.1203/00006450-197902000-00008
PMID:432006
Abstract

Glycine transport in human diploid fibroblasts was shown to be by a single sodium-dependent system. Glycine transport does not appear to exhibit transstimulation or transinhibition. Transport appears to be similar to the A transport system of other mammalian cell lines, as defined by competition patterns. Normal and nonketotic hyperglycinemia (NKH) fibroblasts could not be distinguished on the basis of accumulation or initial rates. A distribution ratio of 15 to 30 was reached by both types of cells. The normal lines have slightly lower apparent Kms (1.1-1.3 mM) than the NKH lines (1.8 to 2.4 mM). The values for the Vmax of the normal cells (11.4-12.9 nmole/mg/min) and the NKH cells (7.0-16.7 nmole/mg/min) overlapped. There were no measurable differences in either the long-term incorporation into protein of leucine and glycine or the oxidation of glycine in normal and NKH fibroblasts.

摘要

人类二倍体成纤维细胞中的甘氨酸转运显示是通过单一的钠依赖系统进行的。甘氨酸转运似乎不表现出转刺激或转抑制。根据竞争模式定义,该转运似乎与其他哺乳动物细胞系的A转运系统相似。正常和非酮症高甘氨酸血症(NKH)成纤维细胞在积累或初始速率方面无法区分。两种类型的细胞都达到了15至30的分布比。正常细胞系的表观Km值(1.1 - 1.3 mM)略低于NKH细胞系(1.8至2.4 mM)。正常细胞(11.4 - 12.9纳摩尔/毫克/分钟)和NKH细胞(7.0 - 16.7纳摩尔/毫克/分钟)的Vmax值重叠。在正常和NKH成纤维细胞中,亮氨酸和甘氨酸长期掺入蛋白质或甘氨酸氧化方面均无可测量的差异。

相似文献

1
Glycine transport by human diploid fibroblasts--absence of a defect in cells from patients with nonketotic hyperglycinemia.人二倍体成纤维细胞对甘氨酸的转运——非酮症高甘氨酸血症患者细胞无转运缺陷
Pediatr Res. 1979 Feb;13(2):127-30. doi: 10.1203/00006450-197902000-00008.
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引用本文的文献

1
Glycine transport by cultured skin fibroblasts from a patient with isolated hyperglycinuria.来自一名孤立性高甘氨酸尿症患者的培养皮肤成纤维细胞对甘氨酸的转运。
J Inherit Metab Dis. 1980;3(3):97-8. doi: 10.1007/BF02312539.
2
Normal glycine transport in cultured diploid fibroblasts from hyperglycinaemic subjects.高甘氨酸血症患者培养的二倍体成纤维细胞中的正常甘氨酸转运
J Inherit Metab Dis. 1983;6(2):82-4. doi: 10.1007/BF02338974.
3
Non-ketotic hyperglycinaemia: clinical and biochemical aspects.
Eur J Pediatr. 1987 May;146(3):221-7. doi: 10.1007/BF00716464.