uroporphyrinogen


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uroporphyrinogen

 [u″ro-por″fĭ-rin´o-jen]
a porphyrinogen formed from porphobilinogen; it is a precursor of uroporphyrin and coproporphyrinogen.
Miller-Keane Encyclopedia and Dictionary of Medicine, Nursing, and Allied Health, Seventh Edition. © 2003 by Saunders, an imprint of Elsevier, Inc. All rights reserved.

por·phy·rin·o·gens

(pōr'fi-rin'ō-jenz),
Intermediates in the biosynthesis of heme, as follows: four porphobilinogens condense to form uroporphyrinogens I and III (giving rise to side products uroporphyrins I and III) that are decarboxylated to form coproporphyrinogens I and III (giving rise to side products coproporphyrins I and III); coproporphyrinogen III is oxidized to protoporphyrinogen III (IX), which is then oxidized to form protoporphyrin III (IX) (this last intermediate adds ferrous iron to yield heme); certain porphyrinogens are elevated in certain porphyrias.
Farlex Partner Medical Dictionary © Farlex 2012

por·phy·rin·o·gens

(pōr'fir-in'ō-jenz)
Intermediates in the biosynthesis of heme; certain porphyrinogens are elevated in certain porphyrias.
Medical Dictionary for the Health Professions and Nursing © Farlex 2012
References in periodicals archive ?
It is an autosomal recessive disorder, which results from deficiency of enzyme uroporphyrinogen III cosynthase (or uroporphyrinogen III synthase).
No gene mutation was observed with uroporphyrinogen decarboxylase ( UROD ) gene analysis.
In the exceedingly rare congenital erythropoietic porphyria (uroporphyrinogen III synthetase deficiency), the stools and urine turn dark red (porphyrinuria) and bones and teeth acquire a yellow-orange color.
Caption: Figure 1: The heme synthetic pathway showing the enzymes mediating formation of delta-ALA, PBG, and the initial porphyrins (uroporphyrinogen), respectively.
The most common of these rare disorders is porphyria cutanea tarda (PCT) caused by an activity deficit of the fifth enzyme in the heme synthetic pathway uroporphyrinogen decarboxylase (UROD) [4].
Further researches on the characteristics of chlorophyll metabolism indicated that the precursors of D-aminolevulinic acid (ALA), porphobilinogen (PBG), uroporphyrinogen III (Urogen III), coproporphyrinogen III (Coprogen III), protoporphyrin IX (Proto IX), Mg-protoporphyrin IX (Mg-Proto IX) and protochlorophyllide (Pchlide) in chlorophyll biosynthesis in Burley21 were lower than in Maryland609 at vigorous growing period; the activity of D-aminolevulinate dehydratase (ALAD) in Burley21 was 0.43% as compared to Maryland609, but the activity of chlorophyllase in Burley21 was 2.04 times as high as in that of Maryland609.
In both the classical and the alternative pathway, 5-aminolevulinic acid (ALA) serves as the common precursor for heme formation which is converted into the biosynthetic intermediate uroporphyrinogen III (UROGEN) in three consecutive enzymatic steps.
Chen, "Uroporphyrinogen decarboxylase as a potential target for specific components of traditional Chinese medicine: a virtual screening and molecular dynamics study," PLoS ONE, vol.
[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.
Rios de Molina, "Comparison between crab hepatopancreas and rat liver uroporphyrinogen decarboxylase," Comparative Biochemistry and Physiology B, vol.
Congenital erythropoietic porphyria, also known as Gunther disease, is one of the first inborn errors of metabolisms to be described.3 This autosomal recessive disorder is caused by deficient activity of uroporphyrinogen III synthase (URO IIIs)/ uroporphyrinogen cosynthase in the erythrocyte precursor cells.