[迟发性皮肤卟啉组:在血红素生物合成酶缺陷中的作用]。

A Adrien, G Guillet
{"title":"[迟发性皮肤卟啉组:在血红素生物合成酶缺陷中的作用]。","authors":"A Adrien,&nbsp;G Guillet","doi":"","DOIUrl":null,"url":null,"abstract":"<p><p>Porphyria cutanea tarda (PCT) is the most common porphyria. In contrast to the other porphyrias, which are undisputably hereditary, PCT was long considered to be acquired. However, the recent demonstration of a defect in uroporphyrinogen decarboxylase suggests that PCT is supported by a genetic factor. Though the hereditary or acquired nature of the enzymatic defect is still under debate, it seems that the disease has a polygenic origin involving an impairment of iron metabolism and a specific genetic background.</p>","PeriodicalId":18005,"journal":{"name":"La semaine des hopitaux : organe fonde par l'Association d'enseignement medical des hopitaux de Paris","volume":"60 16","pages":"1148-52"},"PeriodicalIF":0.0000,"publicationDate":"1984-04-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"[The porphyria cutanea tarda group: their role among the enzyme defects of heme biosynthesis].\",\"authors\":\"A Adrien,&nbsp;G Guillet\",\"doi\":\"\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Porphyria cutanea tarda (PCT) is the most common porphyria. In contrast to the other porphyrias, which are undisputably hereditary, PCT was long considered to be acquired. However, the recent demonstration of a defect in uroporphyrinogen decarboxylase suggests that PCT is supported by a genetic factor. Though the hereditary or acquired nature of the enzymatic defect is still under debate, it seems that the disease has a polygenic origin involving an impairment of iron metabolism and a specific genetic background.</p>\",\"PeriodicalId\":18005,\"journal\":{\"name\":\"La semaine des hopitaux : organe fonde par l'Association d'enseignement medical des hopitaux de Paris\",\"volume\":\"60 16\",\"pages\":\"1148-52\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"1984-04-12\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"La semaine des hopitaux : organe fonde par l'Association d'enseignement medical des hopitaux de Paris\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"La semaine des hopitaux : organe fonde par l'Association d'enseignement medical des hopitaux de Paris","FirstCategoryId":"1085","ListUrlMain":"","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0

摘要

迟发性皮肤卟啉症(PCT)是最常见的卟啉症。与其他卟啉症相比,这是无可争议的遗传,PCT长期以来被认为是获得性的。然而,最近显示的尿卟啉原脱羧酶缺陷表明PCT是由遗传因素支持的。尽管酶缺陷的遗传或获得性仍在争论中,但似乎该疾病具有多基因起源,涉及铁代谢损伤和特定的遗传背景。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[The porphyria cutanea tarda group: their role among the enzyme defects of heme biosynthesis].

Porphyria cutanea tarda (PCT) is the most common porphyria. In contrast to the other porphyrias, which are undisputably hereditary, PCT was long considered to be acquired. However, the recent demonstration of a defect in uroporphyrinogen decarboxylase suggests that PCT is supported by a genetic factor. Though the hereditary or acquired nature of the enzymatic defect is still under debate, it seems that the disease has a polygenic origin involving an impairment of iron metabolism and a specific genetic background.

求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
自引率
0.00%
发文量
0
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信