Automated description from the Alliance of Genome Resources (Release 7.1.0)
Enables uroporphyrinogen-III synthase activity. Involved in heme A biosynthetic process; heme B biosynthetic process; and heme O biosynthetic process. Acts upstream of or within porphyrin-containing compound biosynthetic process. Located in mitochondrion. Is active in cytosol. Is expressed in several structures, including genitourinary system; nervous system; respiratory system; sensory organ; and skeleton. Used to study cutaneous porphyria. Human ortholog(s) of this gene implicated in cutaneous porphyria. Orthologous to human UROS (uroporphyrinogen III synthase).