Tbio PNPT1 Polyribonucleotide nucleotidyltransferase 1, mitochondrial

Protein Summary

Description

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. 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 Accession IDs

• Q8TCS8
• Q53SU0
• Q68CN1
• Q7Z7D1
• Q8IWX1
• Q96T05
• Q9BRU3
• Q9BVX0

Gene Name

• PNPT1

Ensembl ID

• ENST00000415374
• ENSP00000393953
• ENSG00000138035
• ENST00000447944
• ENSP00000400646

Symbol

• PNPASE
• OLD35
• DFNB70
• PNPASE
• old-35
• COXPD13

Knowledge Table

Most Knowledge About	Knowledge Value (0 to 1 scale)
protein domain	1
virus perturbation	1
biological process	0.93
molecular function	0.91
disease perturbation	0.83

Protein Classes

PANTHER Classes

Protein / Transferase / Nucleotidyltransferase / Polyribonucleotide Nucleotidyltransferase 1, Mitochondrial

Protein / Nucleic Acid Binding / Nuclease / Exoribonuclease / Polyribonucleotide Nucleotidyltransferase 1, Mitochondrial

Protein / Nucleic Acid Binding / Rna Binding Protein / Exoribonuclease / Polyribonucleotide Nucleotidyltransferase 1, Mitochondrial

Protein / Hydrolase / Esterase / Polyribonucleotide Nucleotidyltransferase 1, Mitochondrial

DTO Classes

Protein / Enzyme / Hydrolase / Esterase / Polyribonucleotide Nucleotidyltransferase 1, Mitochondrial

IDG Development Level Summary

Tdark

These are targets about which virtually nothing is known. They do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 333.77   (req: < 5)

Gene RIFs: 42   (req: <= 3)

Antibodies: 293   (req: <= 50)

Tbio

These targets do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 333.77   (req: >= 5)

Gene RIFs: 42   (req: > 3)

Antibodies: 293   (req: > 50)

- OR - satisfy the following criterion:

Gene Ontology Terms: 33

Tchem

Target has at least one ChEMBL compound with an activity cutoff of < 30 nM - AND - satisfies the preceding conditions

Active Ligand: 0

Tclin

Target has at least one approved drug - AND - satisfies the preceding conditions

Active Drug: 0