PNPT1 Antibody – Rabbit Polyclonal

PNPT1: RNA-binding protein implicated in numerous RNA metabolic processes. Hydrolyzes single-stranded polyribonucleotides processively in the 3'-to-5' direction. Mitochondrial intermembrane factor with RNA-processing exoribonulease activity. 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. Required for correct processing and polyadenylation of mitochondrial mRNAs. Plays a role as a cytoplasmic RNA import factor that mediates the translocation of small RNA components, like the 5S RNA, the RNA subunit of ribonuclease P and the mitochondrial RNA-processing (MRP) RNA, into the mitochondrial matrix. Plays a role in mitochondrial morphogenesis and respiration; regulates the expression of the electron transport chain (ETC) components at the mRNA and protein levels. In the cytoplasm, shows a 3'-to-5' exoribonuclease mediating mRNA degradation activity; degrades c- myc mRNA upon treatment with IFNB1/IFN-beta, resulting in a growth arrest in melanoma cells. Regulates the stability of specific mature miRNAs in melanoma cells; specifically and selectively degrades miR-221, preferentially. Plays also a role in RNA cell surveillance by cleaning up oxidized RNAs. Binds to the RNA subunit of ribonuclease P, MRP RNA and miR-221 microRNA. Belongs to the polyribonucleotide nucleotidyltransferase family.

Protein type: EC 2.7.7.8; Mitochondrial; Nucleotide Metabolism - purine; Nucleotide Metabolism - pyrimidine; RNA-binding; Transferase

Chromosomal Location of Human Ortholog: 2p16.1

Cellular Component: cytoplasm; cytosol; mitochondrial intermembrane space; mitochondrion

Molecular Function: 3'-5'-exoribonuclease activity; miRNA binding; poly(rG) binding; poly(U) binding; polyribonucleotide nucleotidyltransferase activity; protein binding; RNA binding

Biological Process: mRNA catabolic process; negative regulation of growth; protein homooligomerization; regulation of cellular respiration; RNA catabolic process; RNA polyadenylation

Disease: Combined Oxidative Phosphorylation Deficiency 13; Deafness, Autosomal Recessive 70