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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。

Glycine transport by cultured skin fibroblasts from a patient with isolated hyperglycinuria.

作者信息

Fénéant M, Moatti N, Lemonnier F, Maccario J, Gautier M, Charpentier C, Lemonnier A

出版信息

J Inherit Metab Dis. 1980;3(3):97-8. doi: 10.1007/BF02312539.

DOI:10.1007/BF02312539
PMID:6775149
Abstract

Glycine transport is studied in cultured skin fibroblasts from a patient with isolated hyperglycinuria and from five normal subjects. Fibroblasts from the patient take up glycine less well than do cell lines from controls. Kinetic studies are consistent with a single transport system in the patient's and controls' cell lines. Vmax value in the hyperglycinuric lines is normal, but the apparent affinity is always reduced as opposed to those of four different control lines separately tested. Statistical analysis shows significant difference between Km values.

摘要

对一名患有孤立性高甘氨酸尿症的患者以及五名正常受试者的培养皮肤成纤维细胞中的甘氨酸转运进行了研究。该患者的成纤维细胞摄取甘氨酸的能力不如对照组的细胞系。动力学研究表明,患者和对照组细胞系中存在单一转运系统。高甘氨酸尿症细胞系中的Vmax值正常,但与分别测试的四个不同对照组细胞系相比,其表观亲和力总是降低。统计分析表明Km值之间存在显著差异。

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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.
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Protein measurement with the Folin phenol reagent.使用福林酚试剂进行蛋白质测定。
J Biol Chem. 1951 Nov;193(1):265-75.
2
Transport of dibasic amino acids, cystine, and tryptophan by cultured human fibroblasts: absence of a defect in cystinuria and Hartnup disease.培养的人成纤维细胞对二碱基氨基酸、胱氨酸和色氨酸的转运:胱氨酸尿症和哈特纳普病无缺陷
J Clin Invest. 1972 Aug;51(8):2130-42. doi: 10.1172/JCI107020.
3
Transport of L-isoleucine by cultured human fibroblasts. Uptake by normal cell lines and isolation of a cell line lacking sodium-dependent uptake.
培养的人成纤维细胞对L-异亮氨酸的转运。正常细胞系的摄取以及缺乏钠依赖性摄取的细胞系的分离。
J Biol Chem. 1974 Jun 10;249(11):3430-5.
4
Lowe's syndrome. Absence of amino acid transport defect in cultured fibroblasts.洛氏综合征。培养的成纤维细胞中氨基酸转运缺陷缺失。
Acta Paediatr Scand. 1973 Nov;62(6):615-6. doi: 10.1111/j.1651-2227.1973.tb17073.x.
5
Transport of amino acids by confluent and nonconfluent 3T3 and polyoma virus-transformed 3T3 cells growing on glass cover slips.在玻璃盖玻片上生长的汇合和未汇合的3T3细胞以及多瘤病毒转化的3T3细胞对氨基酸的转运。
J Biol Chem. 1969 May 25;244(10):2675-81.
6
Glycine transport in normal and non-ketotic hyperglycinemic human diploid fibroblasts.正常及非酮症高甘氨酸血症人二倍体成纤维细胞中的甘氨酸转运
Exp Cell Res. 1976 Jun;100(1):95-103. doi: 10.1016/0014-4827(76)90331-1.
7
Effect of cycloleucine on amino acid accumulation by human diploid fibroblasts.
Clin Chim Acta. 1978 Jun 15;86(3):245-9. doi: 10.1016/0009-8981(78)90378-9.
8
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
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