Uroporphyrinogen III synthase
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| Uroporphyrinogen-III synthase | |||||||||
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| Lua error in package.lua at line 80: module 'Module:InfoboxImage/data' not found. Uroporphyrinogen-III synthase monomer, Thermus thermophilus | |||||||||
| Identifiers | |||||||||
| EC no. | 4.2.1.75 | ||||||||
| CAS no. | Template:CAS | ||||||||
| Databases | |||||||||
| IntEnz | IntEnz view | ||||||||
| BRENDA | BRENDA entry | ||||||||
| ExPASy | NiceZyme view | ||||||||
| KEGG | KEGG entry | ||||||||
| MetaCyc | metabolic pathway | ||||||||
| PRIAM | profile | ||||||||
| PDB structures | RCSB PDB PDBe PDBsum | ||||||||
| Gene Ontology | AmiGO / QuickGO | ||||||||
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| Uroporphyrinogen III synthase | |||||||
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| Identifiers | |||||||
| Symbol | UROS | ||||||
| NCBI gene | 7390 | ||||||
| HGNC | 12592 | ||||||
| OMIM | 606938 | ||||||
| RefSeq | NM_000375 | ||||||
| UniProt | P10746 | ||||||
| Other data | |||||||
| EC number | 4.2.1.75 | ||||||
| Locus | Chr. 10 q25.2-26.3 | ||||||
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| Uroporphyrinogen-III synthase HemD | |||||||
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| Lua error in package.lua at line 80: module 'Module:InfoboxImage/data' not found. crystal structure of uroporphyrinogen iii synthase from an extremely thermophilic bacterium thermus thermophilus hb8 (wild type, native, form-2 crystal) | |||||||
| Identifiers | |||||||
| Symbol | HEM4 | ||||||
| Pfam | PF02602 | ||||||
| InterPro | IPR003754 | ||||||
| SCOP2 | 1jr2 / SCOPe / SUPFAM | ||||||
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Uroporphyrinogen III synthase (Template:EnzExplorer) is an enzyme involved in the metabolism of the cyclic tetrapyrrole compound porphyrin. It is involved in the conversion of hydroxymethylbilane into uroporphyrinogen III. This enzyme catalyses the inversion of the final pyrrole unit (ring D) of the linear tetrapyrrole molecule, linking it to the first pyrrole unit (ring A), thereby generating a large macrocyclic structure, uroporphyrinogen III.[1] The enzyme folds into two alpha/beta domains connected by a beta-ladder, the active site being located between the two domains.[2]
Function
The enzyme catalyses the cyclisation reaction of hydroxymethylbilane into uroporphyrinogen III via a spiro intermediate which allows one of the pyrrole rings to convert its initial acetate to propionate configuration into a propionate-acetate one.[3][4]
Pathology
A deficiency is associated with Gunther's disease, also known as congenital erythropoietic porphyria (CEP). This is an autosomal recessive inborn error of metabolism that results from the markedly deficient activity of uroporphyrinogen III synthase.[5]
References
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- ^ Page Module:Citation/CS1/styles.css has no content.Raux E, Schubert HL, Warren MJ (December 2000). "Biosynthesis of cobalamin (vitamin B12): a bacterial conundrum". Cell. Mol. Life Sci. 57 (13–14): 1880–93. doi:10.1007/PL00000670. PMC 11147154. PMID 11215515. S2CID 583311.
- ^ Page Module:Citation/CS1/styles.css has no content.Mathews MA, Schubert HL, Whitby FG, Alexander KJ, Schadick K, Bergonia HA, Phillips JD, Hill CP (November 2001). "Crystal structure of human uroporphyrinogen III synthase". EMBO J. 20 (21): 5832–9. doi:10.1093/emboj/20.21.5832. PMC 125291. PMID 11689424.
- ^ Page Module:Citation/CS1/styles.css has no content.Battersby, Alan R.; Fookes, Christopher J. R.; Matcham, George W.J.; McDonald, Edward (1980). "Biosynthesis of the pigments of life: formation of the macrocycle". Nature. 285 (5759): 17–21. Bibcode:1980Natur.285...17B. doi:10.1038/285017a0. PMID 6769048. S2CID 9070849.
- ^ Enzyme 4.2.1.75 at KEGG Pathway Database.
- ^ Page Module:Citation/CS1/styles.css has no content.To-Figueras J, Badenas C, Mascaro JM, Madrigal I, Merino A, Bastida P, Lecha M, Herrero C (2007). "Study of the genotype-phenotype relationship in four cases of congenital erythropoietic porphyria". Blood Cells Mol. Dis. 38 (3): 242–6. doi:10.1016/j.bcmd.2006.12.001. PMID 17270473.
External links
- Uroporphyrinogen+III+synthase at the U.S. National Library of Medicine Medical Subject Headings (MeSH)
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