Dong-Sun Lee et al.
Proceedings of the National Academy of Sciences of the United States of America, 102(40), 14232-14237 (2005-09-24)
Hereditary coproporphyria is an autosomal dominant disorder resulting from the half-normal activity of coproporphyrinogen oxidase (CPO), a mitochondrial enzyme catalyzing the antepenultimate step in heme biosynthesis. The mechanism by which CPO catalyzes oxidative decarboxylation, in an extraordinary metal- and cofactor-independent