Protein only RNase P catalytic subunit (PRORP)

PRORP, or Protein only RNase P catalytic subunit (also known as MRPP3), is a mitochondrial enzyme that functions as the catalytic core of the mitochondrial RNase P complex. Its primary biological function is to catalyze the precise 5'-end processing of mitochondrial precursor tRNAs (pre-tRNAs), an essential step for the maturation of tRNAs and subsequent mitochondrial protein synthesis. Unlike its bacterial and nuclear counterparts, mitochondrial RNase P in humans is a protein-only complex. Mutations in the gene encoding PRORP are linked to severe mitochondrial disorders, such as combined oxidative phosphorylation deficiency (COXPD), particularly COXPD54, characterized by neurological deficits, hypotonia, and lactic acidosis, highlighting its critical role in mitochondrial gene expression and overall mitochondrial health.

Other names

Mitochondrial ribonuclease P catalytic subunitKIAA0391MRPP3Mitochondrial RNase P protein 3Mitochondrial ribonuclease P protein 3Mitochondrial RNase P subunit 3Proteinaceous RNase PCOXPD54