Article
Structural insight into human variegate porphyria disease.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology - 1 Feb 2011
Qin Xiaohong, Tan Ying, Wang Lele, Wang Zhifang, Wang Baifan, Wen Xin, Yang Guangfu, Xi Zhen, Shen Yuequan
Abstract excerpt
Human protoporphyrinogen IX oxidase (hPPO), a mitochondrial inner membrane protein, converts protoporphyrinogen IX to protoporphyrin IX in the heme biosynthetic pathway. Mutations in the hPPO gene cause the inherited human disease variegate porphyria (VP). In this study, we report the crystal structure of hPPO in complex with the coenzyme flavin adenine dinucleotide (FAD) and the inhibitor acifluorfen at a...
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