InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: PNPT1

Summary

InnateDB Protein

IDBP-376583.4

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

PNPT1

Protein Name

polyribonucleotide nucleotidyltransferase 1

Synonyms

Species

Homo sapiens

Ensembl Protein

ENSP00000393953

InnateDB Gene

IDBG-52626 (PNPT1)

Protein Structure

UniProt Annotation

Function

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. {ECO:0000269PubMed:12473748, ECO:0000269PubMed:12721301, ECO:0000269PubMed:12798676, ECO:0000269PubMed:16055741, ECO:0000269PubMed:16410805, ECO:0000269PubMed:16934922, ECO:0000269PubMed:18083836, ECO:0000269PubMed:18083837, ECO:0000269PubMed:18501193, ECO:0000269PubMed:19509288, ECO:0000269PubMed:20547861, ECO:0000269PubMed:20691904}.

Subcellular Localization

Cytoplasm. Mitochondrion. Mitochondrion intermembrane space; Peripheral membrane protein.

Disease Associations

Combined oxidative phosphorylation deficiency 13 (COXPD13) [MIM:614932]: A mitochondrial disorder characterized by early onset severe encephalomyopathy, dystonia, choreoathetosis, bucofacial dyskinesias and combined mitochondrial respiratory chain deficiency. Nerve conductions velocities are decreased. Levels of plasma and cerebrospinal fluid lactate are increased. {ECO:0000269PubMed:23084291}. Note=The disease is caused by mutations affecting the gene represented in this entry.Deafness, autosomal recessive, 70 (DFNB70) [MIM:614934]: A form of non-syndromic deafness characterized by severe, bilateral hearing impairment with prelingual onset, resulting in inability to acquire normal speech. {ECO:0000269PubMed:23084290}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 18 experimentally validated interaction(s) in this database.

Experimentally validated
Total	18 [view]
Protein-Protein	18 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0000175	3'-5'-exoribonuclease activity
GO:0003676	nucleic acid binding
GO:0003723	RNA binding
GO:0004654	polyribonucleotide nucleotidyltransferase activity
GO:0005515	protein binding
GO:0008266	poly(U) RNA binding
GO:0034046	poly(G) binding
GO:0035198	miRNA binding
GO:0044822	poly(A) RNA binding

Biological Process

GO:0000957	mitochondrial RNA catabolic process
GO:0000958	mitochondrial mRNA catabolic process
GO:0000962	positive regulation of mitochondrial RNA catabolic process
GO:0000964	mitochondrial RNA 5'-end processing
GO:0000965	mitochondrial RNA 3'-end processing
GO:0006396	RNA processing
GO:0006401	RNA catabolic process
GO:0006402	mRNA catabolic process
GO:0034599	cellular response to oxidative stress
GO:0035458	cellular response to interferon-beta
GO:0035927	RNA import into mitochondrion
GO:0035928	rRNA import into mitochondrion
GO:0043457	regulation of cellular respiration
GO:0043631	RNA polyadenylation
GO:0045926	negative regulation of growth
GO:0051260	protein homooligomerization
GO:0061014	positive regulation of mRNA catabolic process
GO:0070207	protein homotrimerization
GO:0070584	mitochondrion morphogenesis
GO:0071042	nuclear polyadenylation-dependent mRNA catabolic process
GO:0071850	mitotic cell cycle arrest
GO:0090503	RNA phosphodiester bond hydrolysis, exonucleolytic
GO:0097222	mitochondrial mRNA polyadenylation
GO:2000627	positive regulation of miRNA catabolic process
GO:2000772	regulation of cellular senescence

Cellular Component

GO:0005737	cytoplasm
GO:0005739	mitochondrion
GO:0005758	mitochondrial intermembrane space
GO:0016020	membrane
GO:0045025	mitochondrial degradosome

Protein Structure and Domains

PDB ID

InterPro

IPR001247 Exoribonuclease, phosphorolytic domain 1
IPR003029 Ribosomal protein S1, RNA-binding domain
IPR004087 K Homology domain
IPR004088 K Homology domain, type 1
IPR012162 Polyribonucleotide nucleotidyltransferase
IPR012340 Nucleic acid-binding, OB-fold
IPR015847 Exoribonuclease, phosphorolytic domain 2
IPR015848 Polyribonucleotide nucleotidyltransferase, RNA-binding domain
IPR020568 Ribosomal protein S5 domain 2-type fold
IPR022967 RNA-binding domain, S1

PFAM

PF01138
PF00575
PF00013
PF13014
PF03725
PF03726

PRINTS

PIRSF

PIRSF005499

SMART

SM00322
SM00316

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt