Automated description from the Alliance of Genome Resources (Release 7.0.0)
Enables gamma-butyrobetaine dioxygenase activity. Involved in carnitine biosynthetic process. Located in mitochondrion. Is active in cytosol. Is expressed in several structures, including alimentary system; genitourinary system; nervous system; respiratory system; and retina. Orthologous to human BBOX1 (gamma-butyrobetaine hydroxylase 1).