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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).
仅用于科研。不用于诊断过程。未经明确授权不得转售。
蛋白别名: 3'-5' RNA exonuclease OLD35; DKFZp762K1914; old 35; PNPase 1; PNPase old-35; Polynucleotide phosphorylase 1; Polynucleotide phosphorylase-like protein; Polyribonucleotide nucleotidyltransferase 1, mitochondrial
基因别名: COXPD13; DFNB70; old-35; OLD35; PNPASE; PNPT1
UniProt ID: (Human) Q8TCS8
Entrez Gene ID: (Human) 87178