Target intelligence / Profile preview

Zinc phosphodiesterase ELAC protein 2 (ELAC2)

Target
ELAC2
Molecular classification
Enzyme, Metallo-β-lactamase family, Endoribonuclease
01

Overview

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].

Other names
elaC ribonuclease Z 2RNase Z 2tRNA 3 endonuclease 2tRNase Z 2ELAC2HPC2FLJ10530elaC homolog protein 2heredity prostate cancer protein 2ELC2COXPD17ElaC-like protein 2putative prostate cancer susceptibility protein HPC2/ELAC2
02

Biological functions

tRNA 3′ processing (3′-trailer removal from pre-tRNA)tRNA maturationRegulation of mitochondrial RNA metabolism
03

Disease associations

Cancer (notably hereditary prostate cancer)Cardiovascular disease (hypertrophic cardiomyopathy)Mitochondrial disorders
04

Safety considerations

Loss-of-function mutations may cause impaired tRNA processing, mitochondrial dysfunction, and increased risk for cancer or cardiomyopathy
05

Biomarkers

Mutations in ELAC2 for hereditary prostate cancer risk stratificationMutations in ELAC2 for hypertrophic cardiomyopathy diagnosis

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