UROPORPHYRINOGEN DECARBOXYLASE

被引：69

作者：

ELDER, GH

ROBERTS, AG

机构：

[1] Department of Medical Biochemistry, University of Wales College of Medicine, Cardiff

来源：

JOURNAL OF BIOENERGETICS AND BIOMEMBRANES | 1995年 / 27卷 / 02期

关键词：

UROPORPHYRINOGEN DECARBOXYLASE; PORPHYRIA; IRON; HEME BIOSYNTHESIS; UROPORPHYRINOGEN; CYTOCHROME P450IA; HEPATOERYTHROPOIETIC PORPHYRIA; PORPHYRIA CUTANEA TARDA;

D O I：

10.1007/BF02110035

中图分类号：

Q6 [生物物理学];

学科分类号：

071011 ;

摘要：

Uroporphyrinogen decarboxylase (EC 4.1.1.37) catalyzes the decarboxylation of uroporphyrinogen III to coproporphyrinogen III. The amino acid sequences, kinetic properties, and physicochemical characteristics of enzymes from different sources (mammals, yeast, bacteria) are similar, but little is known about the structure/function relationships of uroporphyrinogen decarboxylases. Halogenated and other aromatic hydrocarbons cause hepatic uroporphyria by decreasing hepatic uroporphyrinogen decarboxylase activity. Two related human porphyrias, porphyria cutanea tarda and hepatoerythropoietic porphyria, also result from deficiency of this enzyme. The roles of inherited and acquired factors, including iron, in the pathogenesis of human and experimental uroporphyrias are reviewed.

引用

页码：207 / 214

页数：8

共 87 条

[1] PORPHYRIA-CUTANEA-TARDA AND HLA-LINKED HEMOCHROMATOSIS - ALL IN THE FAMILY [J].