Acute intermittent porphyria - port wine coloured urine?

[goal270]

Why is there port wine colored urine in AIP, if the enzyme blocked is before formation of uroporphyrinogen?

Also, why do cytochrome p450 inducers precipitate AIP?

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[faisal 2000]

Also, why do cytochrome p450 inducers precipitate AIP?

δ-aminolevulinic acid catalyzed by ALA synthase. as you know acute intermittent porphyria occurs as result of accumulation of δ-aminolevulinic acid. when we use p450 inducers which will derepressing δ-aminolevulenic acid synthetase that will leads to Precipitation of acute intermittent porphyria. it also precipitated by barbiturates and ethanol.

 

[LyMed]

p450 enzymes contain heme. When you induce p450, you induce the heme pathway and precipitate any deficiency.