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• W2097773417 endingPage "14237" @default.
• W2097773417 startingPage "14232" @default.
• W2097773417 abstract "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 manner, is poorly understood. Here, we report the crystal structure of human CPO at 1.58-Å resolution. The structure reveals a previously uncharacterized tertiary topology comprising an unusually flat seven-stranded β-sheet sandwiched by α-helices. In the biologically active dimer ( K D = 5 × 10 -7 M), one monomer rotates relative to the second by ≈40° to create an intersubunit interface in close proximity to two independent enzymatic sites. The unexpected finding of citrate at the active site allows us to assign Ser-244, His-258, Asn-260, Arg-262, Asp-282, and Arg-332 as residues mediating substrate recognition and decarboxylation. We favor a mechanism in which oxygen serves as the immediate electron acceptor, and a substrate radical or a carbanion with substantial radical character participates in catalysis. Although several mutations in the CPO gene have been described, the molecular basis for how these alterations diminish enzyme activity is unknown. We show that deletion of residues (392-418) encoded by exon six disrupts dimerization. Conversely, harderoporphyria-causing K404E mutation precludes a type I β-turn from retaining the substrate for the second decarboxylation cycle. Together, these findings resolve several questions regarding CPO catalysis and provide insights into hereditary coproporphyria." @default.
• W2097773417 created "2016-06-24" @default.
• W2097773417 creator A5054673621 @default.
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• W2097773417 creator A5067253674 @default.
• W2097773417 creator A5073092312 @default.
• W2097773417 creator A5028898852 @default.
• W2097773417 date "2005-09-21" @default.
• W2097773417 modified "2023-10-10" @default.
• W2097773417 title "Structural basis of hereditary coproporphyria" @default.
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• W2097773417 doi "https://doi.org/10.1073/pnas.0506557102" @default.
• W2097773417 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/1224704" @default.
• W2097773417 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/16176984" @default.
• W2097773417 hasPublicationYear "2005" @default.
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