uroporphyrinogen III cosynthase

ur·o·por·phy·rin·o·gen III co·syn·thase

an enzyme in heme biosynthesis that participates in the formation of uroporphyrinogen III; a deficiency of this protein results in congenital erythropoietic porphyria.
Mentioned in ?
References in periodicals archive ?
1,2] Primary abnormality in CEP is due to decreased uroporphyrinogen III cosynthase activity resulting in accumulation and hyperexcretion of biologically inactive type I porphyrins.
CEP is due to deficiency of the enzyme uroporphyrinogen III cosynthase (UIIIC).