PNPT1 blocking peptide

PNPT1 Antibody (Center) Blocking Peptide

• Gene Names: PNPT1; OLD35; DFNB70; PNPASE; old-35; COXPD13
• Synonyms: PNPT1; PNPT1 Antibody (Center) Blocking Peptide; Polyribonucleotide nucleotidyltransferase 1; mitochondrial; 3'-5' RNA exonuclease OLD35; PNPase old-35; Polynucleotide phosphorylase 1; PNPase 1; Polynucleotide phosphorylase-like protein; PNPASE; PNPT1 blocking peptide

• Form/Format: Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.
• Sequence Length: 783

• Cellular Location: Cytoplasm. Mitochondrion. Mitochondrion intermembrane space; Peripheral membrane protein
• Preparation and Storage: Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.

Related Product Information for PNPT1 blocking peptide

RNA-binding protein implicated in numerous RNA metabolic processes. Catalyzes the phosphorolysis of 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.

NCBI and Uniprot Product Information

• NCBI GI #: 115502437
• NCBI GeneID: 87178
• UniProt Accession #: Q8TCS8; Q53SU0; Q68CN1; Q7Z7D1; Q8IWX1; Q96T05; Q9BRU3; Q9BVX0
• Molecular Weight: 85,951 Da
• NCBI Official Full Name: Polyribonucleotide nucleotidyltransferase 1, mitochondrial
• NCBI Official Synonym Full Names: polyribonucleotide nucleotidyltransferase 1
• NCBI Official Symbol: PNPT1
• NCBI Official Synonym Symbols: OLD35; DFNB70; PNPASE; old-35; COXPD13
• NCBI Protein Information: polyribonucleotide nucleotidyltransferase 1, mitochondrial
• UniProt Protein Name: Polyribonucleotide nucleotidyltransferase 1, mitochondrial
• UniProt Gene Name: PNPT1
• UniProt Synonym Gene Names: PNPASE; PNPase 1
• UniProt Entry Name: PNPT1_HUMAN

NCBI Description

The protein encoded by this gene belongs to the evolutionary conserved polynucleotide phosphorylase family comprised of phosphate dependent 3'-to-5' exoribonucleases implicated in RNA processing and degradation. This enzyme is predominantly localized in the mitochondrial intermembrane space and is involved in import of RNA to mitochondria. Mutations in this gene have been associated with combined oxidative phosphorylation deficiency-13 and autosomal recessive nonsyndromic deafness-70. Related pseudogenes are found on chromosomes 3 and 7. [provided by RefSeq, Dec 2012]

Uniprot Description

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

Chromosomal Location of Human Ortholog: 2p15

Cellular Component: cytoplasm; mitochondrial intermembrane space; mitochondrion

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