uroporphyrinogen


Also found in: Wikipedia.

uroporphyrinogen

 [u″ro-por″fĭ-rin´o-jen]
a porphyrinogen formed from porphobilinogen; it is a precursor of uroporphyrin and coproporphyrinogen.

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.

uroporphyrinogen

/uro·por·phy·rin·o·gen/ (-por″fĭ-rin´o-jen) a porphyrinogen formed from porphobilinogen; it is a precursor of uroporphyrin and coproporphyrinogen.

por·phy·rin·o·gens

(pōr'fir-in'ō-jenz)
Intermediates in the biosynthesis of heme; certain porphyrinogens are elevated in certain porphyrias.

uroporphyrinogen

a precursor of uroporphyrin and coproporphyrinogen.

uroporphyrinogen III
first tetrapyrrole compound in the pathway of porphyrin synthesis from combination of four porphobilinogen compounds via the action of uroporphyrinogen I synthetase and uroporphyrinogen III cosynthetase.
uroporphyrinogen III cosynthetase
an enzyme involved in the synthesis of uroporphyrinogen.
uroporphyrinogen isomerase
see uroporphyrinogen III cosynthetase (above).
uroporphyrinogen I synthetase
an enzyme involved in the synthesis of uroporphyrinogen.
References in periodicals archive ?
No gene mutation was observed with uroporphyrinogen decarboxylase ( UROD ) gene analysis.
Uroporphyrinogen 3 cosynthetase activity in the fox squirrel (Sciurus niger).
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.
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.
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.
3 This autosomal recessive disorder is caused by deficient activity of uroporphyrinogen III synthase (URO IIIs)/ uroporphyrinogen cosynthase in the erythrocyte precursor cells.
6 Uroporphyrinogen, which accumulates in PCT, appears not to share this ability.
Porphyria cutanea tarda, hepatitis C, uroporphyrinogen decarboxylase and mutations of HFE gene.
Acute intermittent porphyria (AIP) is a hereditary hepatic porphyria inherited as autosomal dominant with low penetrance resulting from mutation in the gene encoding the enzyme, porphobilinogen deaminase (PBG-D) [hydroxymethylbilane synthase, uroporphyrinogen I synthase].
In EP, a deficient activity of the enzyme uroporphyrinogen III synthase in erythrocyte precursor cells results in overproduction of isomer I porphyrinogens that cannot be used to form heme.