Coproporphyrinogen-III oxidase, mitochondrial (abbreviated as CPOX) is an enzyme that in humans is encoded by the CPOX gene. (wikipedia.org)
CPOX, the sixth enzyme of the haem biosynthetic pathway, converts coproporphyrinogen III to protoporphyrinogen IX through two sequential steps of oxidative decarboxylation. (wikipedia.org)
CPOX is an enzyme involved in the sixth step of porphyrin metabolism it catalyses the oxidative decarboxylation of coproporphyrinogen III to proto-porphyrinogen IX in the haem and chlorophyll biosynthetic pathways. (wikipedia.org)
The coproporphinogen oxidase gene is a enzyme that converts coproporphyrinogen III to protoporphyrinogen IX (OMIM). (bionity.com)
Hereditary coproporphyria is the result of a point mutation in the coproporphinogen oxidase (CPO) gene (OMIM). (bionity.com)
Publications] Shigeru Sassa: 'Molecular cletects of the coproporphyrinogen oxidase gene in hereditary coproporphyria. (nii.ac.jp)
Variegate porphyria (VP) is an inherited disorder of porphyrin-heme metabolism arising from mutations of the gene encoding the enzyme protoporphyrinogen oxidase. (medscape.com)
Variegate porphyria arises from autosomal dominant inheritance of a gene mutation encoding a defective protoporphyrinogen oxidase enzyme. (medscape.com)
Prevalence is estimated at 1 case in 300 persons in South Africa, where a protoporphyrinogen oxidase gene "founder" mutation traceable to Dutch immigrants who married there in 1680 has been widely disseminated. (medscape.com)
This relatively high prevalence compared with Europe suggests a possible protoporphyrinogen oxidase gene mutation passed down through Jewish ancestry. (medscape.com)
Analysis of the mechanism underlying a mild phenotype of hereditary coproporphyria due to a homozygous missense mutation in the transcription initiation codon of the coproporphyrinogen III oxidase gene. (cdc.gov)
However, with early diagnosis of active disease, identification of asymptomatic protoporphyrinogen oxidase gene mutation carriers, and avoidance of drugs and other factors known to induce or worsen clinical expression, symptomatology among those at risk can be minimized. (medscape.com)
Enzyme4
As only patients with mutation in this region (K404E) would develop harderoporphyria, this mutation led to diminishment of the second step of the decarboxylation reaction during the conversion of coproporphyrinogen to protoporphyrinogen, implying that the active site of the enzyme involved in the second step of decarboxylation is located in exon 6. (wikipedia.org)
Coproporphyrinogen oxidase (CPO) is an essential enzyme that catalyzes the sixth step of the heme biosynthetic pathway. (rcsb.org)
Hereditary coproporphyria (HCP) is a form of hepatic porphyria associated with a deficiency of the enzyme coproporphyrinogen III oxidase. (bionity.com)
Inheritance of two mutant protoporphyrin oxidase genes causes a more profound reduction in residual enzyme activity to 25% or less, leading to more severe disease manifestations. (medscape.com)
Heme2
The Escherichia coli Protein YfeX Functions as a Porphyrinogen Oxidase, Not a Heme Dechelatase. (porphyria.org)
Lead causes a decrease in heme synthesis by inhibiting Delta-aminolevulinic acid (delta-ALA) dehydratase, ferrochelatase, and the coproporphyrinogen oxidase pathway. (picmonic.com)
The last three enzymes of the pathway, namely, coproporphyrinogen III oxidase, protoporphyrinogen oxidase, and ferrochelatase, are also located within mitochondria, whereas the 2nd-5th enzymes are found in the cytoplasm or soluble fraction of the cell. (ashpublications.org)
Defect1
Cause is partial defect in coproporphyrin oxidase with deficient conversion of coproporphyrinogen-9. (insidesurgery.com)
Substrate2
Substrate specificity of catechol oxidase from Lycopus europaeus and characterization of the bioproducts of enzymic caffeic acid oxidation. (lookformedical.com)
The substrate specificity of catechol oxidase from Lycopus europaeus towards phenols is examined. (lookformedical.com)
Function1
[ 2 ] In patients with coproporphyria, the function of coproporphyrinogen oxidase is only 40-60% of normal. (medscape.com)
In vitro metabolism of citalopram by monoamine oxidase B in human blood. (ncats.io)
Enzyme coproporphyrinogen oxidase1
It is caused due to the deficiency of the enzyme coproporphyrinogen oxidase. (targetwoman.com)
Heme3
The enzyme catalyzes the step-wise oxidative decarboxylation of the heme precursor, coproporphyrinogen III, to protoporphyrinogen IX via a tricarboxylic intermediate, harderoporphyrinogen. (nih.gov)
During heme biosynthesis the oxygen-independent coproporphyrinogen III oxidase HemN catalyzes the oxidative decarboxylation of the two propionate side chains on rings A and B of coproporphyrinogen III to the corresponding vinyl groups to yield protoporphyrinogen IX. (uni-hannover.de)
This enzyme catalyzes the stepwise oxidative decarboxylation of coproporphyrinogen III to protoporphyrinogen IX, a precursor of heme. (nih.gov)
Conversion of coproporphyrinogen1
As only patients with mutation in this region (K404E) would develop harderoporphyria, this mutation led to diminishment of the second step of the decarboxylation reaction during the conversion of coproporphyrinogen to protoporphyrinogen, implying that the active site of the enzyme involved in the second step of decarboxylation is located in exon 6. (wikipedia.org)
Protoporphyrinogen oxidase then catalyzes the oxygen-dependent aromatization of protoporphyrinogen into protoporphyrin. (pougetlaw.com)
Porphyria1
Variegate porphyria (also porphyria variegata or mixed porphyria ) results from a partial deficiency in PROTO oxidase, manifesting itself with skin lesions similar to those of porphyria cutanea tarda combined with acute neurologic attacks. (the-medical-dictionary.com)
Oxidative decarboxylation3
An enzyme that catalyzes the oxidative decarboxylation of coproporphyrinogen III to protoporphyrinogen IX by the conversion of two propionate groups to two vinyl groups. (bvsalud.org)
Catalyzes the aerobic oxidative decarboxylation of propionate groups of rings A and B of coproporphyrinogen-III to yield the vinyl groups in protoporphyrinogen-IX. (nih.gov)
The next step is the oxidative decarboxylation of the propionate side chains on the ring A and B of coproporphyrinogen molecule to yield protoporphyrinogen IX. (pougetlaw.com)
MRNA1
Simultaneously, steady-state levels of coproporphyrinogen oxidase mRNA increased but aminolaevulinic acid dehydratase mRNA levels remained unchanged. (nih.gov)
Genetic1
Modification of neurobehavioral effects of mercury by a genetic polymorphism of coproporphyrinogen oxidase in children. (nih.gov)
Genes1
Inheritance of two mutant protoporphyrin oxidase genes causes a more profound reduction in residual enzyme activity to 25% or less, leading to more severe disease manifestations. (medscape.com)