Deybach J C, Da Silva V, Phung L N, Levy J C, Nordmann Y
Presse Med. 1987 Jan 24;16(2):68-71.
Hepatic porphyrias (acute intermittent porphyria, coproporphyria, porphyria variegata and porphyria cutanea) are inherited diseases affecting porphyrin-heme metabolism in the liver. A situation analogous to the latent stage of human hepatic porphyria can be produced in chick embryos by injecting an inhibitor of ferrochelatase (the last enzyme in the heme biosynthesis pathway); the simultaneous injection of a porphyrinogenic drug (e.g. barbiturate) results in a strong increase of liver porphyrin levels, as is found in human hepatic porphyrias. This experimental model facilitates drug screening: drugs that are found to be porphyrinogenic in ovo are potentially dangerous to patients with hepatic porphyria and should be avoided.
肝性卟啉病(急性间歇性卟啉病、粪卟啉病、混合型卟啉病和迟发性皮肤卟啉病)是影响肝脏中卟啉 - 血红素代谢的遗传性疾病。通过向鸡胚注射亚铁螯合酶抑制剂(血红素生物合成途径中的最后一种酶),可以产生类似于人类肝性卟啉病潜伏期的情况;同时注射一种致卟啉药物(如巴比妥酸盐)会导致肝脏卟啉水平大幅升高,这与人类肝性卟啉病的情况相同。这种实验模型有助于药物筛选:在鸡胚中被发现有致卟啉作用的药物对肝性卟啉病患者可能具有潜在危险性,应予以避免。