Crystal structure of human uroporphyrinogen III synthase

EMBO Journal

Volumn 20, Issue 21, 2001, Pages 5832-5839

  Mathews, Michael A A ^a   Schubert, Heidi L ^a   Whitby, Frank G ^a   Alexander, Kelly J ^a   Schadick, Kevin ^a   Bergonia, Hector A ^a   Phillips, John D ^a   Hill, Christopher P ^a  

^a UNIVERSITY OF UTAH SCHOOL OF MEDICINE   (United States)

Author keywords

Cobalamin; Congenital erythropoietic porphyria; Heme; Porphyrin; Uroporphyrinogen

Indexed keywords

CHLOROPHYLL; CYANOCOBALAMIN; HEME; HEME DERIVATIVE; PYRROLE DERIVATIVE; UROPORPHYRINOGEN; UROPORPHYRINOGEN III SYNTHASE;

ARTICLE; AUTOSOMAL RECESSIVE DISORDER; CATALYSIS; CONGENITAL ERYTHROPOIETIC PORPHYRIA; CRYSTAL STRUCTURE; CYCLIZATION; ENZYME ACTIVE SITE; ENZYME ACTIVITY; ENZYME STRUCTURE; HUMAN; HUMAN CELL; MUTATION; NUCLEOTIDE SEQUENCE; PRIORITY JOURNAL; PROTEIN DOMAIN; PROTEIN FOLDING;

BINDING SITES; CRYSTALLOGRAPHY, X-RAY; HUMANS; MODELS, MOLECULAR; MOLECULAR SEQUENCE DATA; MUTAGENESIS, SITE-DIRECTED; MUTATION; PORPHYRIA, ERYTHROPOIETIC; PROTEIN STRUCTURE, SECONDARY; PROTEIN STRUCTURE, TERTIARY; RECOMBINANT PROTEINS; SEQUENCE HOMOLOGY, AMINO ACID; STRUCTURE-ACTIVITY RELATIONSHIP; UROPORPHYRINOGEN III SYNTHETASE;

EID: 0035502972     PISSN: 02614189     EISSN: None     Source Type: Journal    
DOI: 10.1093/emboj/20.21.5832     Document Type: Article

References (35)

1. The discovery of nature's biosynthetic pathways
2. Ideas and experiments in biosynthesis
3. Biosynthesis of the pigments of life: Mechanistic studies on the conversion of porphobilinogen to uroporphyrinogen III
4. Biosynthesis of type-III porphyrins: Proof of intact enzymatic conversion of the head-to-tail bilane into uro'gen III by intramolecular arrangement
5. Ribbons 2.0
6. Synthetic studies on the proposed spiro intermediate for biosynthesis of the natural porphyrins: The stereochemical probe
7. The CCP4 suite: Programs for protein crystallography
8. Density modification for macromolecular phase improvement
9. Maximum-likelihood heavy-atom parameter refinement in the MIR and MAD methods
10. Molecular genetics of congenital erythropoietic porphyria
11. 12-binding domains of methionine synthase
12. Accurate bond and angle parameters for X-ray protein structure refinement
13. Fast and sensitive multiple sequence alignments on a microcomputer
14. Dali/FSSP classification of three-dimensional protein folds
15. Improved methods for binding protein models in electron density maps and the location of errors in these models
16. Dictionary of protein secondary structure: Pattern recognition of hydrogen-bonded and geometrical features
17. The porphyrias
18. PROCHECK: A program to check the stereochemical quality of protein structures
19. The evidence for a spirocyclic intermediate in the formation of uroporphyrinogen III by cosynthase
20. Biosynthesis of pyrrolepigments: A mechanism for porphybilinogen polymerization
21. Efficient anisotropic refinement of macromolecular structures using FFT
22. SCOP: A structural classification of proteins database for the investigation of sequences and structures
23. Oscillation data reduction program
24. Evidence for an intermediate in the enzymatic formation of uroporphyrinogen III
25. Studies on uro'gen III synthase with modified bilanes
26. Crystal structure of globular domain of histone H5 and its implications for nucleosome binding
27. Congenital erythropoietic porphyria: A novel homozygous mutation in a Japanese patient
28. Congenital erythropoietic porphyria affecting two brothers
29. 12 - A survey of the last four billion years
30. Synthetic studies on the proposed spiro intermediate for biosynthesis of the natural porphyrins: Inhibition of cosynthase
31. Novel point mutation in the uroporphyrinogen III synthase gene causes congenital erythropoietic porphyria of a Japanese family
32. Automated MAD and MIR structure solution
33. Purification and properties of uroporphyrinogen III synthase from human erythrocytes
34. Human uroporphyrinogen III synthase: Molecular cloning, nucleotide sequence, and expression of a full-length cDNA
35. Congenital erythropoietic porphyria: Identification and expression of exonic mutations in the uroporphyrinogen III synthase gene