uroporphyrinogen decarboxylase

ur·o·por·phy·rin·o·gen de·car·box·yl·ase

an enzyme that participates in heme biosynthesis; it catalyzes the decarboxylation of uroporphyrin III to produce coproporphyrinogen III; it also acts on uroporphyrin I; a deficiency of this enzyme will result in either porphyria cutanea tarda or hepatoerythropoietic porphyria.
References in periodicals archive ?
No gene mutation was observed with uroporphyrinogen decarboxylase ( UROD ) gene analysis.
Herrero, "Identification and characterization of novel uroporphyrinogen decarboxylase gene mutations in a large series of porphyria cutanea tarda patients and relatives," Clinical Genetics, vol.
The mRNAs included control housekeeping genes [actin, beta (ACTB) [4] and beta-2-microglobulin (B2M)] and myeloid [defensin, alpha 3, neutrophil-specific (DEFA3) and serglycin (SRGN)], erythroid [hemoglobin, beta (HBB) and uroporphyrinogen decarboxylase (UROD)], and megakaryocyte [integrin, alpha 2b (platelet glycoprotein IIb of IIb/IIIa complex, antigen CD41) (ITGA2B) and integrin, beta 3 (platelet glycoprotein IIIa, antigen CD61) (ITGB3)] lineage-specific genes.
Chen, "Uroporphyrinogen decarboxylase as a potential target for specific components of traditional Chinese medicine: a virtual screening and molecular dynamics study," PLoS ONE, vol.
Rios de Molina, "Comparison between crab hepatopancreas and rat liver uroporphyrinogen decarboxylase," Comparative Biochemistry and Physiology B, vol.
We have studied patients with congenital erythropoietic porphyria and hepato-erythropoietic porphyria, arising from double defects in the enzymes uroporphyrinogen cosynthase and uroporphyrinogen decarboxylase respectively, as well as homozygous VP.
1991) showing that precoproporphyrin is formed as a consequence of Hg inhibition of uroporphyrinogen decarboxylase in the kidney during prolonged exposure, producing excess pentacarboxylporphyrinogen, which then competes with coproporphyrinogen as a substrate for coproporphyrinogen oxidase as the basis of precoproporphyrin formation (Woods et al.
Porphyria cutanea tarda, hepatitis C, uroporphyrinogen decarboxylase and mutations of HFE gene.
In most cases, the PCT that occurs in patients with HCV is acquired due to a decrease in functional hepatic uroporphyrinogen decarboxylase, she said.
[7] Nonstandard abbreviations: EPNET, European Porphyria Network; ALA, [delta]-aminolevulinic acid; PBG, porphobilinogen; EQAS, external quality assessment scheme; [TE.sub.a], total allowable error; EPP, erythropoietic protoporphyria; PCT, porphyria cutanea tarda; PBGD, porphobilinogen deaminase; UROD, uroporphyrinogen decarboxylase; AIP, acute intermittent porphyria; VP, variegate porphyria; HCP, hereditary coproporphyria; CIII:I, coproporphyrin isomer III:I.
These include aminolevulinate (ALA) dehydratase, porphobilinogen deaminase (hydroxymethylbilane synthase), and uroporphyrinogen decarboxylase in spleen and ALA synthase in liver.