Bovine protoporphyria: the first nonhuman model of this hereditary photosensitizing disease.
Bovine protoporphyria: the first nonhuman model of this hereditary photosensitizing disease.
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牛原卟啉症：这种遗传性光敏性疾病的第一个非人类模型。
DOI：
10.1126/science.905823
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发表时间：
1977
期刊：
影响因子：
56.9
通讯作者：
B. Stephenson
中科院分区：
文献类型：
--
作者：
G. Ruth;S. Schwartz;B. Stephenson
原卟啉症是一种只在人类中记录的光敏性疾病，它以隐性性状传播给7头雌性小牛。皮肤病变是广泛的，红细胞和粪便中的原卟啉浓度远远超过人类原卟啉症。平均亚铁螯合酶活性降低到一半的正常的携带者的肝脏，和约十分之一的正常的肝，肾，心脏，脾，肺，骨髓的原卟啉。
Protoporphyria, a photosensitizing disease documented only in humans, was transmitted as a recessive trait to seven female calves. Cutaneous lesions were extensive, and erythrocyte and fecal protoporphyrin concentrations exceeded by far those of human protoporphyria. Average ferrochelatase activity was decreased to one-half of normal in the liver of carriers, and to about one-tenth of normal in liver, kidney, heart, spleen, lung, and marrow of protoporphyrics.