Hepatic porphyria
Encyclopedia
Hepatic porphyrias is a form of porphyria
in which the enzyme deficiency occurs in the liver
.
Examples include (in order of synthesis pathway):
Porphyria
Porphyrias are a group of inherited or acquired disorders of certain enzymes in the heme bio-synthetic pathway . They are broadly classified as acute porphyrias and cutaneous porphyrias, based on the site of the overproduction and accumulation of the porphyrins...
in which the enzyme deficiency occurs in the liver
Liver
The liver is a vital organ present in vertebrates and some other animals. It has a wide range of functions, including detoxification, protein synthesis, and production of biochemicals necessary for digestion...
.
Examples include (in order of synthesis pathway):
- Acute intermittent porphyriaAcute intermittent porphyriaAcute intermittent porphyria is a rare autosomal dominant metabolic disorder affecting the production of heme, the oxygen-binding prosthetic group of hemoglobin. It is characterized by a deficiency of the enzyme porphobilinogen deaminase. Acute intermittent porphyria is the second most common...
- Porphyria cutanea tardaPorphyria cutanea tardaPorphyria cutanea tarda is the most common subtype of porphyria. 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...
and Hepatoerythropoietic porphyriaHepatoerythropoietic porphyriaHepatoerythropoietic porphyria is a very rare form of hepatic porphyria caused by a disorder in both genes which code Uroporphyrinogen III decarboxylase .It has a similar presentation to porphyria cutanea tarda , but with earlier onset... - Hereditary coproporphyriaHereditary coproporphyriaHereditary coproporphyria is a type of acute porphyria that is associated with a deficiency of the enzyme coproporphyrinogen III oxidase....
- Variegate porphyriaVariegate porphyriaVariegate porphyria is an autosomal dominant porphyria that can have acute symptoms along with symptoms that affect the skin...
External links
- www.drugs-porphyria.com
- www.porphyria-europe.com