porphyrinogen

[por″fĭ-rin´o-jen]

the reduced form of a porphyrin. The porphyrinogens are the functional intermediates in the biosynthesis of heme and if oxidized to their corresponding porphyrins, such as occurs in porphyrias, are irreversibly removed from the biosynthetic pathway and accumulate in tissues. Their nomenclature corresponds to that of the porphyrins.

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We were interested to find in this study that fecal coproporphyrin was a uniformly better diagnostic marker for VP than protoporphyrin, although the predominant porphyrinogen excreted in bile in individuals with VP is protoporphyrinogen (24).

Plasma fluorescence scanning and fecal porphyrin analysis for the diagnosis of variegate porphyria: precise determination of sensitivity and specificity with detection of protoporphyrinogen oxidase mutations as a reference standard

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.

Red urine and photosensitive skin rash: not only was our patient's urine red, but his teeth had a reddish hue, as well

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