Mitochondrial RNase P (mtRNase P) cleaves tRNA molecules in their 5'-ends and consists of 3 components: a tRNA methyltransferase (MRPP1/TRMT10), a dehydrogenase/reductase-family member (MRPP2/HSD17B10), and MRPP3 (KIAA03910) [].This entry represents tRNA methyltransferase 10 homologue C (TRMT10C), which is a metazoa homologue to yeast TRM10, a tRNA m1G methyltransferase responsible for modification at position 9. In humans there are three TRM10 orthologues. Mutations in TRMT10C cause mitochondrial disease [].