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  2. Porphyria - Wikipedia

    en.wikipedia.org/wiki/Porphyria

    Porphyria / p ɔːr ˈ f ɪr i ə / is a group of disorders in which substances called porphyrins build up in the body, adversely affecting the skin or nervous system. [1] The types that affect the nervous system are also known as acute porphyria, as symptoms are rapid in onset and short in duration. [1]

  3. Porphyria cutanea tarda - Wikipedia

    en.wikipedia.org/wiki/Porphyria_cutanea_tarda

    Porphyria cutanea tarda is the most common subtype of porphyria. [1] The disease is named because it is a porphyria that often presents with skin manifestations later in life. The disorder results from low levels of the enzyme responsible for the fifth step in heme production. Heme is a vital molecule for all of the body's organs.

  4. Acute intermittent porphyria - Wikipedia

    en.wikipedia.org/wiki/Acute_intermittent_porphyria

    Acute intermittent porphyria (AIP) is a rare metabolic disorder affecting the production of heme resulting from a deficiency of the enzyme porphobilinogen deaminase. It is the most common of the acute porphyrias .

  5. Erythropoietic protoporphyria - Wikipedia

    en.wikipedia.org/wiki/Erythropoietic_protoporphyria

    Erythropoietic protoporphyria (or commonly called EPP) is a form of porphyria, which varies in severity and can be very painful.It arises from a deficiency in the enzyme ferrochelatase, leading to abnormally high levels of protoporphyrin in the red blood cells (erythrocytes), plasma, skin, and liver. [2]

  6. Variegate porphyria - Wikipedia

    en.wikipedia.org/wiki/Variegate_porphyria

    Variegate porphyria has an autosomal dominant pattern of inheritance. Mutations in the PPOX gene cause variegate porphyria. [4] The PPOX gene makes a membrane bound mitochondrial enzyme called protoporphyrinogen oxidase, which is critical to the chemical process that leads to heme production. The activity of this enzyme is reduced by 50 percent ...

  7. Pseudoporphyria - Wikipedia

    en.wikipedia.org/wiki/Pseudoporphyria

    A personal and family history of hepatitis, porphyria, or photosensitivity disorder must be sought. Although a genetic factor has not been considered in pseudoporphyria, one case of monozygotic twins developing pseudoporphyria after excessive UV-A exposure from long-term tanning bed use has been documented.

  8. Erythropoietic porphyria - Wikipedia

    en.wikipedia.org/wiki/Erythropoietic_porphyria

    X-linked sideroblastic anemia or "X-linked dominant erythropoietic protoporphyria", associated with ALAS2 (aminolevulinic acid synthase), has also been described.X-linked dominant erythropoietic protoporphyria (XDEPP) is caused by a gain of function mutation in the ALAS2 (5-aminolevulinate synthase) gene; that gene encodes the very first enzyme in the heme biosynthetic pathway.

  9. Porphobilinogen deaminase - Wikipedia

    en.wikipedia.org/wiki/Porphobilinogen_deaminase

    The most well-known health issue involving porphobilinogen deaminase is acute intermittent porphyria, an autosomal dominant genetic disorder where insufficient hydroxymethylbilane is produced, leading to a build-up of porphobilinogen in the cytoplasm. This is caused by a gene mutation that, in 90% of cases, causes decreased amounts of enzyme.

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