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Atlas of Genetics and Cytogenetics in Oncology and Haematology OPEN ACCESS JOURNAL AT INIST-CNRS Gene Section Mini Review NONO non-POU domain containing, octamerbinding Jean-Loup Huret Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France (JLH) Published in Atlas Database: January 1999 Online updated version : http://AtlasGeneticsOncology.org/Genes/NONOID168.html DOI: 10.4267/2042/37499 This work is licensed under a Creative Commons Attribution-Noncommercial-No Derivative Works 2.0 France Licence. © 1999 Atlas of Genetics and Cytogenetics in Oncology and Haematology Identity active; NONO also enhances the binding of several DNA-binding proteins (but not all). HGNC (Hugo): NONO Location: Xq12 Homology With PSF and other proteins with a DBHS domain (Drosophila behaviour, human splicing) which includes the tandem RNA binding domains. DNA/RNA Transcription Implicated in Alternative splicing; 2.4 mRNA complete cds; coding sequence: CDS 129 ... 1550. inv(X)(p11.2q12) in renal cell carcinoma --> NonO/TFE3 Protein Disease Only one case of papillary renal cell carcinoma. Hybrid/Mutated gene 5' NONO - 3' TFE3. Description The protein encoded by NONO is called p54nrb (nuclear RNA binbing); 471 amino acids; 54 kDa; Nterm glutamine/histidine rich region, tandem RNA binding domains (amino acids 75 to 228), helix-turnhelix implicated in DNA binding, and a proline rich region in C-term; N-term and C-term allow proteinprotein interactions. 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. Expression References Wide. Function Dong B, Horowitz DS, Kobayashi R, Krainer AR. 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. Nucleic Acids Res. 1993 Aug 25;21(17):4085-92 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 Atlas Genet Cytogenet Oncol Haematol. 1999; 3(2) 54 NONO non-POU domain containing, octamer-binding Huret JL Yang YS, Hanke JH, Carayannopoulos L, Craft CM, Capra JD, Tucker PW. NonO, a non-POU-domain-containing, octamerbinding protein, is the mammalian homolog of Drosophila nonAdiss. Mol Cell Biol. 1993 Sep;13(9):5593-603 Yang YS, Yang MC, Tucker PW, Capra JD. NonO enhances the association of many DNA-binding proteins to their targets. Nucleic Acids Res. 1997 Jun 15;25(12):2284-92 Straub T, Grue P, Uhse A, Lisby M, Knudsen BR, Tange TO, Westergaard O, Boege F. The RNA-splicing factor PSF/p54 controls DNA-topoisomerase I activity by a direct interaction. J Biol Chem. 1998 Oct 9;273(41):26261-4 Clark J, Lu YJ, Sidhar SK, Parker C, Gill S, Smedley D, Hamoudi R, Linehan WM, Shipley J, Cooper CS. Fusion of splicing factor genes PSF and NonO (p54nrb) to the TFE3 gene in papillary renal cell carcinoma. Oncogene. 1997 Oct;15(18):2233-9 This article should be referenced as such: Huret JL. NONO non-POU domain containing, octamerbinding. Atlas Genet Cytogenet Oncol Haematol. 1999; 3(2):54-55. Atlas Genet Cytogenet Oncol Haematol. 1999; 3(2) 55