Atlas of Genetics and Cytogenetics in Oncology and Haematology
NONO
| Identity |
| HGNC (Hugo) | NONO |
| Location | Xq12 |
| Location_base_pair | Starts at 70419767 and ends at 70437741 bp from pter ( according to hg18-Mar_2006) [Mapping] |
| DNA/RNA |
| Transcription | alternative splicing; 2.4 mRNA complete cds; coding sequence: CDS 129 ... 1550 |
| Protein |
 | |
| Description | the protein encoded by NONO is called p54nrb (nuclear RNA binbing); 471 amino acids; 54 kDa; N-term glutamine/histidine rich region, tandem RNA binding domains (amino acids 75 to 228), helix-turn-helix implicated in DNA binding, and a proline rich region in C-term; N-term and C-term allow protein-protein interactions. |
| Expression | wide |
| Function | binds independantly to DNA and RNA; forms a heterodimer with PSF, a protein sharing vast homologies; both form complexes with DNA topoisomerase I, which renders this enzyme much more active; NONO also enhances the binding of several DNA-binding proteins (but not all). |
| Homology | with PSF and other proteins with a DBHS domain (Drosophila behaviour, human splicing) which includes the tandem RNA binding domains. |
| Implicated in |
| Entity | inv(X)(p11.2q12) in renal cell carcinoma -->NonO/TFE3 |
| Disease | only one case of papillary renal cell carcinoma |
 | |
| Hybrid/Mutated Gene | 5' NONO- 3' TFE3 |
| Abnormal Protein | N-term NONO and most of it except the C-term proline rich sequence fused to the DNA binding domains of TFE3 (excluding the acidic transcriptional activation domain, including the C-term helix-loop-helix, and the leucine zipper); the reciprocal transcript is found |
| External links |
| Bibliography |
| Purification and cDNA cloning of HeLa cell p54nrb, a nuclear protein with two RNA recognition motifs and extensive homology to human splicing factor PSF and Drosophila NONA/BJ6. |
| Dong B, Horowitz DS, Kobayashi R, Krainer AR |
| Nucleic acids research. 1993 ; 21 (17) : 4085-4092. |
| PMID 8371983 |
| NonO, a non-POU-domain-containing, octamer-binding protein, is the mammalian homolog of Drosophila nonAdiss. |
| Yang YS, Hanke JH, Carayannopoulos L, Craft CM, Capra JD, Tucker PW |
| Molecular and cellular biology. 1993 ; 13 (9) : 5593-5603. |
| PMID 8355702 |
| Fusion of splicing factor genes PSF and NonO (p54nrb) to the TFE3 gene in papillary renal cell carcinoma. |
| Clark J, Lu YJ, Sidhar SK, Parker C, Gill S, Smedley D, Hamoudi R, Linehan WM, Shipley J, Cooper CS |
| Oncogene. 1997 ; 15 (18) : 2233-2239. |
| PMID 9393982 |
| NonO enhances the association of many DNA-binding proteins to their targets. |
| Yang YS, Yang MC, Tucker PW, Capra JD |
| Nucleic acids research. 1997 ; 25 (12) : 2284-2292. |
| PMID 9171077 |
| The RNA-splicing factor PSF/p54 controls DNA-topoisomerase I activity by a direct interaction. |
| Straub T, Grue P, Uhse A, Lisby M, Knudsen BR, Tange TO, Westergaard O, Boege F |
| The Journal of biological chemistry. 1998 ; 273 (41) : 26261-26264. |
| PMID 9756848 |
| REVIEW articles | automatic search in PubMed |
| Last year publications | automatic search in PubMed |
| Contributor(s) |
| Written | 01-1999 | Jean-Loup Huret |
| Citation |
| This paper should be referenced as such : |
| Huret JL . NONO. Atlas Genet Cytogenet Oncol Haematol. January 1999 . URL : http://AtlasGeneticsOncology.org/Genes/NONOID168.html |
| © Atlas of Genetics and Cytogenetics in Oncology and Haematology | indexed on : Sat Jun 27 16:39:24 CEST 2009 |
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