PNPT1 (polyribonucleotide nucleotidyltransferase 1, mitochondirla) is a RNA-binding protein implicated in numerous RNA metabolic processes. It catalyzes the phosphorolysis of single-stranded polyribonucleotides processively in the 3'-to-5' direction. PNPT1 is a component of the mitochondrial degradosome (mtEXO) complex that degrades 3' overhang double-stranded RNA with a 3'-to-5' directionality in an ATP-dependent manner. It is required for correct processing and polyadenylation of mitochondrial mRNAs. It plays a role as a cytoplasmic RNA import factor, in mitochondrial morphogenesis and respiration, regulation of expression of the electron transport chain, regulation of the stability of specific mature miRNAs in melanoma cells, and RNA cell surveilance. Mutations of the gene can result in combined oxidative phosphorylation deficiency 13 (COXPD13) and deafness, autosomal recessive, 70 (DFNB70).
3'-5' RNA exonuclease OLD35; DKFZp762K1914; old 35; PNPase 1; PNPase old-35; Polynucleotide phosphorylase 1; Polynucleotide phosphorylase-like protein; Polyribonucleotide nucleotidyltransferase 1, mitochondrial