Article
Structural insight into acute intermittent porphyria.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology - 1 Feb 2009
Song Gaojie, Li Yang, Cheng Chongyun, Zhao Yu, Gao Ang, Zhang Rongguang, Joachimiak Andrzej, Shaw Neil, Liu Zhi-Jie
Abstract excerpt
Acute intermittent porphyria (AIP), an inherited disease of heme biosynthesis, is one of the most common types of porphyria. Reduced activity of the enzyme porphobilinogen deaminase (PBGD), which catalyzes the sequential condensation of 4 molecules of porphobilinogen to yield preuroporphyrinogen, has been linked to the symptoms of AIP. We have determined the 3-dimensional structure of human PBGD at 2.2 A...
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