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抗坏血酸降低培养的胱氨酸病成纤维细胞的游离胱氨酸含量。

Decrease in free cystine content of cultured cystinotic fibroblasts by ascorbic acid.

作者信息

Kroll W A, Schneider J A

出版信息

Science. 1974 Dec 13;186(4168):1040-2. doi: 10.1126/science.186.4168.1040.

DOI:10.1126/science.186.4168.1040
PMID:4469696
Abstract

The 100-fold increase in free cystine content characteristic of cultured skin fibroblasts from patients with nephropathic cystinosis was decreased more than 50 percent by addition of L-ascorbic acid to the culture medium at concentrations of 0.29 to 2.9 millimolar. Fresh ascorbic acid must be added to the culture medium daily to produce a progressive decrease of the free cystine content of the cells over a 3-day period. Upon removal of ascorbic acid from the medium, the free cystine content returns to its initial value.

摘要

肾病性胱氨酸病患者培养的皮肤成纤维细胞中特征性的游离胱氨酸含量增加100倍，在培养基中添加浓度为0.29至2.9毫摩尔的L-抗坏血酸后，该含量降低了50%以上。必须每天向培养基中添加新鲜的抗坏血酸，以便在3天时间内使细胞的游离胱氨酸含量逐渐降低。当从培养基中去除抗坏血酸后，游离胱氨酸含量会恢复到其初始值。

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Cysteamine depletes cystinotic leucocyte granular fractions of cystine by the mechanism of disulphide interchange.半胱胺通过二硫键交换机制消耗胱氨酸病白细胞颗粒部分中的胱氨酸。
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Cystinosis. Intracellular cystine depletion by aminothiols in vitro and in vivo.胱氨酸病。体外和体内通过氨基硫醇使细胞内胱氨酸耗竭。
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Proc R Soc Med. 1977;70 Suppl 3(Suppl 3):37-40. doi: 10.1177/00359157770700S314.

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