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non-POU domain-containing octamer-binding protein; nonO protein; 54 kD nuclear RNA- & DNA-binding protein; 55 kD nuclear protein; DNA-binding p52/p100 complex, 52 kD subunit; NMT55; p54(nrb); p54nrb (NONO, NRB54)

Function: - DNA- & RNA binding protein - involved in several nuclear processes - binds the conventional octamer sequence in double stranded DNA - also binds single-stranded DNA & RNA at a site independent of the duplex site (putative) - involved in pre-mRNA splicing, probably as an heterodimer with SFPQ - interacts with U5 snRNA, probably by binding to a purine-rich sequence located on the 3' side of U5 snRNA stem 1b - the SFPQ-NONO - heteromer associated with MATR3 may play a role in nuclear retention of defective RNAs - the SFPQ-NONO heteromer may be involved in DNA unwinding by modulating the function of topoisomerase I/TOP1 - the SFPQ-NONO heteromer may be involved in DNA nonhomologous end joining (NHEJ) required for double-strand break repair & V(D)J recombination & may stabilize paired DNA ends - in vitro, the complex strongly stimulates DNA end joining, binds directly to the DNA substrates & cooperates with the Ku70/G22P1-Ku80/XRCC5 (Ku) dimer to establish a functional preligation complex - nono is involved in transcriptional regulation - the SFPQ-NONO-NR5A1 complex binds to the CYP17 promoter & regulates basal & cAMP-dependent transcriptional activity - NONO binds to an enhancer element in long terminal repeats of endogenous intracisternal A particles (IAPs) & activates transcription (putative) - monomer & component of the SFPQ-NONO complex, which is probably a heterotetramer of two 52 kD (NONO) & 2 100 kDa (SFPQ) subunits - NONO is a component of spliceosome & U5.4/6 snRNP complexes interacts with PSPC1 & SNRPA/U1A - part of complex consisting of SFPQ, NONO & MATR3 - part of a complex consisting of SFPQ, NONO & NR5A1 - part of a complex consisting of SFPQ, NONO & TOP1 - interacts with SPI1 (putative) - interacts with RNF43 Structure: - - contains 2 RRM domain (RNA recognition motif)s Compartment: nucleus Expression: - expressed in heart, brain, placenta, lung, liver, skeletal muscle, kidney & pancreas - also found in a number of breast tumor cell lines Pathology: - chromosomal translocation t(X;X)(p11.2;q13.1) involving NONO with TFE3 may be a cause of papillary renal cell carcinoma (PRCC)

General

DNA-binding protein RNA-binding protein

Properties

SIZE: entity length = 471 aa MW = 54 kD COMPARTMENT: cell nucleus MOTIF: glutamine-rich region {30-35} MOTIF: glutamine residue (SEVERAL) proline-rich region SITE: 36-42 MOTIF: proline residue (SEVERAL) DBHS {54-373} MOTIF: RNP motif NAME: RNP motif SITE: 74-141 FOR-BINDING-OF: ribonucleic acid MOTIF: ribonucleoprotein-1 motif NAME: ribonucleoprotein-1 motif FOR-BINDING-OF: ribonucleic acid MOTIF: ribonucleoprotein-1 motif ribonucleoprotein-2 motif ribonucleoprotein-2 motif FOR-BINDING-OF: ribonucleic acid MOTIF: ribonucleoprotein-1 motif ribonucleoprotein-2 motif Ser phosphorylation site {S147} RNP motif NAME: RNP motif SITE: 148-229 FOR-BINDING-OF: ribonucleic acid MOTIF: ribonucleoprotein-1 motif NAME: ribonucleoprotein-1 motif FOR-BINDING-OF: ribonucleic acid MOTIF: ribonucleoprotein-1 motif ribonucleoprotein-2 motif ribonucleoprotein-2 motif FOR-BINDING-OF: ribonucleic acid MOTIF: ribonucleoprotein-1 motif ribonucleoprotein-2 motif coiled coil {268-372} arginine-rich region {348-351} MOTIF: arginine residue (SEVERAL) breakpoint {377-378} Thr phosphorylation site {T428} Thr phosphorylation site {T450}

Database Correlations

OMIM 300084 UniProt Q15233 PFAM correlations Entrez Gene 4841 Kegg hsa:4841

References

  1. UniProt :accession Q15233
  2. Atlas of Genetics & Cytogenetics in Oncology & Haematology http://atlasgeneticsoncology.org/genes/NONOID168.html