Enables 3'-tRNA processing endoribonuclease activity. Involved in several processes, including RNA processing; germ cell proliferation; and positive regulation of translation. Located in cytosol; mitochondrion; and nucleus. Is expressed in several structures, including embryonic imaginal tissue; female germline cell; gonad; larval imaginal tissue; and somatic precursor cell. Used to study cardiomyopathy and combined oxidative phosphorylation deficiency 17. Human ortholog(s) of this gene implicated in combined oxidative phosphorylation deficiency 17. Orthologous to human ELAC2 (elaC ribonuclease Z 2).
FB Description
Ribonuclease Z (RNaseZ) is a highly conserved gene that encodes the endoribonuclease RNase Z, which cleaves pre-tRNA molecules directly 3' to the discriminator. It has at least two biological roles - nuclear pre-tRNA processing and mitochondrial primary transcript processing.