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Overview

Uniprot IDQ8K1R3
Protein NamePolyribonucleotide nucleotidyltransferase 1, mitochondrial
Gene NamePnpt1
OrganismMus musculus

Kla Sites from experimental identification

Position Flanking peptide
521 VTKTNPEKGEIEDYR
750 GRMRLSRKVLQSPAT

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. Involved in the degradation of non-coding mitochondrial transcripts (MT-ncRNA) and tRNA-like molecules (By similarity). 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. Also plays 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

Protein Sequence

10 MAACRLCCLC 20 PCLRPLGCGP 30 LGRPGRNRAL 40 SYLQMRALWS 50 STGSRAVTVD 60 LGHRKLEISS 70 GKLARFADGC 80 AVIQSGDTAV 90 MVTAVSKTKA 100 SPSQFMPLVV 110 DYRQKAAAAG 120 RIPTNYLRRE 130 IGSSDREVLT 140 SRVIDRSIRP 150 LFPAGYFYDT 160 QVLCNLLAVD 170 GINEPDILAV 180 NGASVALSLS 190 DIPWNGPVGA 200 VRIGMIDGEC 210 VVNPTRREMS 220 SSTLNLVVAG 230 APKSQIVMLE 240 ASAENILQQD 250 FCHAIKVGVK 260 YTQQIIQGIQ 270 QLVKEIGVAK 280 RTPQKIFTPS 290 AEIVKYTKII 300 AMEKLYAVFT 310 DYEHDKVSRD 320 EAVNKIRLDT 330 EEHLKEKFPE 340 VDQFEIIESF 350 NIVAKEVFRS 360 IILNEYKRCD 370 GRDLTSLRNI 380 SCEVDMFKTL 390 HGSALFQRGQ 400 TQVLCTVTFD 410 SLESSIKSDQ 420 IITAINGVKD 430 KNFMLHYEFP 440 PYATNETGKV 450 TGVNRRELGH 460 GALAEKALCP 470 VIPKDFPFTI 480 RVTSEVLESN 490 GSSSMASACG 500 GSLALMDAGV 510 PISSAVAGVA 520 VGLVTKTNPE 530 KGEIEDYRLL 540 TDILGIEDYN 550 GDMDFKIAGT 560 NKGITALQAD 570 IKLPGVPIKI 580 IMEAIQQASV 590 AKKEILQIMN 600 KTISKPRASR 610 KENGPVVETV 620 KVPLSKRAKF 630 VGPGGYHLKK 640 LQAETGVTIS 650 QVDEETFSIF 660 APTPTAMHEA 670 RDFITEICRD 680 DQEQQLEFGA 690 VYTATITEIR 700 DTGVMVKLYP 710 NMTAVLLHNS 720 QLDQRKIKHP 730 TALGLEVGQE 740 IQVKYFGRDP 750 ADGRMRLSRK 760 VLQSPATTAL 770 KTLNDRSSIV 780 MGEPVSQSSN SNP

Gene Ontology

Classification GO ID Description
Cellular Component GO:0005737 cytoplasm
Cellular Component GO:0005829 cytosol
Cellular Component GO:0005789 endoplasmic reticulum membrane
Cellular Component GO:0045025 mitochondrial degradosome
Cellular Component GO:0005758 mitochondrial intermembrane space
Cellular Component GO:0005759 mitochondrial matrix
Cellular Component GO:0005739 mitochondrion
Cellular Component GO:0005840 ribosome
Molecular Function GO:0000175 3'-5'-RNA exonuclease activity
Molecular Function GO:0042802 identical protein binding
Molecular Function GO:0035198 miRNA binding
Molecular Function GO:0034046 poly(G) binding
Molecular Function GO:0008266 poly(U) RNA binding
Molecular Function GO:0004654 polyribonucleotide nucleotidyltransferase activity
Biological Process GO:0035458 cellular response to interferon-beta
Biological Process GO:0034599 cellular response to oxidative stress
Biological Process GO:0097421 liver regeneration
Biological Process GO:0000958 mitochondrial mRNA catabolic process
Biological Process GO:0097222 mitochondrial mRNA polyadenylation
Biological Process GO:0000965 mitochondrial RNA 3'-end processing
Biological Process GO:0000964 mitochondrial RNA 5'-end processing
Biological Process GO:0000957 mitochondrial RNA catabolic process
Biological Process GO:0007005 mitochondrion organization
Biological Process GO:0006402 mRNA catabolic process
Biological Process GO:0006397 mRNA processing
Biological Process GO:0071042 nuclear polyadenylation-dependent mRNA catabolic process
Biological Process GO:2000627 positive regulation of miRNA catabolic process
Biological Process GO:0000962 positive regulation of mitochondrial RNA catabolic process
Biological Process GO:0061014 positive regulation of mRNA catabolic process
Biological Process GO:0051260 protein homooligomerization
Biological Process GO:0070207 protein homotrimerization
Biological Process GO:0043457 regulation of cellular respiration
Biological Process GO:2000772 regulation of cellular senescence
Biological Process GO:0051591 response to cAMP
Biological Process GO:0060416 response to growth hormone
Biological Process GO:0006401 RNA catabolic process
Biological Process GO:0035927 RNA import into mitochondrion
Biological Process GO:0035928 rRNA import into mitochondrion

Reference

[1] Zhuo W, Zhang M, Tan J, Gao Y, Wang Y et al.. Lysine lactylation analysis of proteins in the heart of the Kawasaki disease mouse model.. Front Cell Dev Biol 13:1550220. 2025. PMID: 40114965.

[2] Wu D, Tang Y, Li X, Xiong S, Zhang Z et al.. Characterization of protein lactylation in healthy and ischemic mouse hearts.. Front Cardiovasc Med 12:1644886. 2025. PMID: 41089239.