UROD : uroporphyrinogen decarboxylase
Description
The UROD (uroporphyrinogen decarboxylase) is a protein-coding gene located on chromosome 1.
The UROD gene provides instructions for making the enzyme uroporphyrinogen decarboxylase, which is essential for heme production. Heme is a vital molecule found in all organs, particularly in the blood, bone marrow, and liver. It is a component of hemoproteins, including hemoglobin, which carries oxygen in the blood. Heme production is a complex process involving eight enzymes. Uroporphyrinogen decarboxylase catalyzes the fifth step, removing carbon and oxygen atoms from uroporphyrinogen III to form coproporphyrinogen III. This reaction is followed by three more enzymatic steps that ultimately lead to the formation of heme.
Uroporphyrinogen decarboxylase (UROD) catalyzes the removal of four acetate side chains from uroporphyrinogen, converting it to coproporphyrinogen. This is the fifth step in the heme biosynthesis pathway. UROD can use either isomer I or isomer III of uroporphyrinogen as a substrate, but only coproporphyrinogen III can be further processed into heme. In laboratory settings, UROD has also been shown to decarboxylate pentacarboxylate porphyrinogen I.
UROD is also known as PCT, UPD.
