ELAC2 (elaC ribonuclease Z 2) is a zinc phosphodiesterase that functions as a mitochondrial tRNase Z, catalyzing 3' end processing of both mitochondrial and nuclear precursor tRNAs 1. In mitochondria, ELAC2 removes 3' trailer sequences from pre-tRNAs as part of multi-step tRNA maturation 2, while associating with mitochondrial nucleoids to coordinate RNA processing and ribosome assembly [UniProt annotation]. ELAC2 operates independently for nuclear pre-tRNA processing but requires accessory proteins TRMT10C and SDR5C1 to process structurally divergent mitochondrial tRNAs lacking canonical features 3. Beyond tRNA processing, ELAC2 participates in degradation of mitochondria-encoded circular RNAs (mecciRNAs) through a complex with SUPV3L1, regulating mitochondrial reactive oxygen species release and permeability transition pore function 4. Clinically, ELAC2 mutations cause combined oxidative phosphorylation deficiency 17, with cardiomyopathy-linked variants demonstrating cardiac hypertrophy and reduced contractility in experimental models 5. ELAC2 polymorphisms, particularly Ser217Leu and Ala541Thr variants, constitute low-penetrance susceptibility markers for hereditary and sporadic prostate cancer 67, with certain polymorphisms associated with elevated PSA levels and tumor staging in affected populations 8.