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Article

Structural basis of hereditary coproporphyria.

Proceedings of the National Academy of Sciences of the United States of America - 4 Oct 2005

Lee Dong-Sun, Flachsová Eva, Bodnárová Michaela, Demeler Borries, Martásek Pavel, Raman C S

Abstract excerpt

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...

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Source

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Identifiers

PMID: 16176984
DOI: 10.1073/pnas.0506557102
PMCID: PMC1224704

Genes

CPO

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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