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铜蓝蛋白基因在成年和新生大鼠肝脏中的表达。

Expression of the caeruloplasmin gene in the adult and neonatal rat liver.

作者信息

Barrow L, Tanner M S, Critchley D R

机构信息

Department of Child Health, University of Leicester, U.K.

出版信息

Clin Sci (Lond). 1989 Sep;77(3):259-63. doi: 10.1042/cs0770259.

Abstract
  1. It has been suggested that low levels of serum caeruloplasmin in Wilson's disease result from the failure to switch from a fetal to an adult mode of caeruloplasmin gene expression. To investigate postnatal expression of the caeruloplasmin gene, steady-state levels of caeruloplasmin messenger RNA in adult and neonatal rat liver were measured. 2. Copper parameters observed in neonatal rats were similar to those seen in Wilson's disease: hepatic copper concentration was significantly elevated (neonatal 164 +/- 35 micrograms/g, adults 50 +/- 8 micrograms/g, P less than .001) and serum copper and caeruloplasmin levels were low (neonatal 0.5 +/- 0.1 microgram/ml, adults 1.3 +/- 0.2 microgram/ml, P less than .001; neonatal 0.20 +/- 0.04 arbitrary units, adults 0.69 +/- 0.16 arbitrary units, P less than .001), respectively. 3. Caeruloplasmin messenger RNA levels were analysed by Northern and dot blotting using a 32P-labelled caeruloplasmin complementary DNA probe. A caeruloplasmin messenger RNA of approximately 4.4 kilobases was detected in both adult and neonatal rat liver, with no significant difference observed in steady-state levels. 4. A step subsequent to caeruloplasmin gene transcription must therefore be impaired in neonatal rats.
摘要
  1. 有人提出,威尔逊氏病患者血清铜蓝蛋白水平低是由于未能从胎儿期铜蓝蛋白基因表达模式转换为成人模式所致。为了研究铜蓝蛋白基因的出生后表达情况,我们检测了成年和新生大鼠肝脏中铜蓝蛋白信使核糖核酸的稳态水平。2. 在新生大鼠中观察到的铜参数与威尔逊氏病患者相似:肝脏铜浓度显著升高(新生大鼠为164±35微克/克,成年大鼠为50±8微克/克,P<0.001),而血清铜和铜蓝蛋白水平则较低(新生大鼠分别为0.5±0.1微克/毫升,成年大鼠为1.3±0.2微克/毫升,P<0.001;新生大鼠为0.20±0.04任意单位,成年大鼠为0.69±0.16任意单位,P<0.001)。3. 使用32P标记的铜蓝蛋白互补DNA探针,通过Northern印迹和斑点印迹分析铜蓝蛋白信使核糖核酸水平。在成年和新生大鼠肝脏中均检测到约4.4千碱基的铜蓝蛋白信使核糖核酸,稳态水平未见显著差异。4. 因此,新生大鼠铜蓝蛋白基因转录后的一个步骤必定受到了损害。

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