briseThe thing that got me screwed was the "increases synthesis of compounds"; like what compounds?+
i_hate_it_hereI swear they just love to say shit to throw us off smh+3
sexymexican888@brise I think they were referring to the previous products of heme synthesis that build up (hydroxymethylbilane, uroporphynogen - or however the fk u spell it) I think those are what cause the blistering photosensitivity, im guessing it is since these dont accumulate in acute intermittent porphyria -> no skin symptoms. but yeah I agree these ppl fucking suck +1
chaosawaits"Disorder caused by the increased synthesis of compounds in the skin that are subject to excitation by visible light" = heme;
"Disorder caused by lack of compounds in the skin that are subject to excitation by visible light" = melanin+1
awkword@sexymexican888 is right. Dr. Ryan says that there's an accumulation of uroporphyrinogen due to lack of UROD which gets oxidized to uroporphyrin. Uroporphyrin then gets transported to the skin (along with being found in plasma and urine), and causes the characteristic skin lesions found when exposed to light.+2
submitted by โicedcoffeeislyfe(56)
FA 2020 pg 425
Porphyria cutanea tarda-- defect in UROD in the heme synthesis pathway that causes photosensitivity and blistering