Dracula is one of the most iconic horror characters, being a vampire who sucked the blood of innocent lives to survive countless centuries. He was deathly pale, sucked blood, afraid of sunlight, and had fangs.
But his characteristics were inspired by a family of genetic mutations called Porphyria. Someone who shows symptoms of porphyria can have sensitivity to light, excessive hair growth, tightening of skin around lips and gums (creating the look of fangs).
Porphyria results from a mutation in genes like ALAD, ALAS2, CPOX, FECH, HMBS, PPOX, UROD, or UROS. These genes code for enzymes that makes a porphyrin called heme, a cofactor in hemoglobin. Heme is produced through a metabolic pathway in the bone marrow and the liver. The mutations in the genes mentioned reduce enzyme activity, resulting in the abnormal buildup of porphyrins in the liver and other organs. For example, Ferrochelatase, is involved in the 8th step of the metabolic pathway in the synthesis of heme. A deficiency of thisenzyme causes EPP (Erythropoietic protoporphyria). The symptoms of EPP can correlate to Dracula's sensitivity to light because one of the most common symptoms is photosensitivity, causing burning and itching.
Porphyria is an inherited disease, but most carriers of the gene don't show symptoms. Those who do, show such symptoms under times of stress and symptoms can flare up through different scenarios such as drinking alcohol or dieting/long periods without food. Treatment of porphyria vary with each disease. Treatments may involve avoiding triggers of symptoms, having heme injected through a vein, or taking blood out to reduce iron int he body. Notable people who have porphyria include Mary, Queen of Scots and King George III. Being that both lived during eras that had no proper medicine, one of the ways to treat their porphyria was through drinking blood, which of course contributed to the Dracula story.
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Is it true that dracula was really based off this mutation or do you believe that? They do have alot of similarities and I can see it being true.
ReplyDeleteI do think he was based off of porphyria but I don't think a usual patient of porphyria would display all the symptoms at the same time like Dracula did (pale skin, low heme, photosensitivity, etc).
ReplyDeleteI feel like I am not reading something right or Im missing it but anyway:
ReplyDeleteHow does drinking blood help "treat" the disease?
It gives the heme they lack.
ReplyDelete