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Zinc phosphodiesterase ELAC protein 2 (ELAC2) is an endoribonuclease enzyme responsible for removing the 3′ trailer from precursor transfer RNA (pre-tRNA) during tRNA maturation. It is a member of the metallo-β-lactamase protein family and contains two conserved β-lactamase domains, with its catalytic activity dependent on a His-motif and two Zn²⁺ ions in the C-terminal domain. ELAC2 is essential for generating mature tRNAs required for protein synthesis in both the nucleus and mitochondria. Mutations in ELAC2 are strongly associated with hereditary prostate cancer and hypertrophic cardiomyopathy. Structural and biochemical studies have revealed conformational flexibility linked to its enzymatic cycle and identified critical regions for substrate binding and catalysis. ELAC2 is thus a biologically essential enzyme with direct links to human disease, but it is not currently known to be directly targeted by any approved therapeutic drugs[1][2][3][4].
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