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NONO (non-POU domain containing, octamer-binding)

Written1999-01Jean-Loup Huret
Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France

(Note : for Links provided by Atlas : click)

Identity

HGNC (Hugo) NONO
HGNC Alias symbNRB54
NMT55
P54NRB
P54
PPP1R114
HGNC Alias nameNuclear RNA-binding protein, 54-kD
 non-Pou domain-containing octamer (ATGCAAAT) binding protein
 protein phosphatase 1, regulatory subunit 114
HGNC Previous namenon-POU-domain-containing, octamer-binding
LocusID (NCBI) 4841
Atlas_Id 168
Location Xq13.1  [Link to chromosome band Xq13]
Location_base_pair Starts at 71283635 and ends at 71301168 bp from pter ( according to GRCh38/hg38-Dec_2013)  [Mapping NONO.png]
Fusion genes
(updated 2017)		Data from Atlas, Mitelman, Cosmic Fusion, Fusion Cancer, TCGA fusion databases with official HUGO symbols (see references in chromosomal bands)
NONO (Xq13.1)::TFE3 (Xp11.23)TFE3 (Xp11.23)::NONO (Xq13.1)

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

Note
  
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
  

Bibliography

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
 
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
 
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
 
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
 
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
 

Citation

This paper should be referenced as such :
Huret, JL
NONO (non-POU domain containing, octamer-binding)
Atlas Genet Cytogenet Oncol Haematol. 1999;3(2):54-55.
Free journal version : [ pdf ]   [ DOI ]

Other Leukemias implicated (Data extracted from papers in the Atlas) [ 4 ]
  t(1;9)(p34;q34) SFPQ::ABL1
t(X;2)(q13;p21) NONO::ZFP36L2
t(X;X)(p11;q13) NONO::TFE3
t(X;X)(p11;q13) TFE3::NONO

External links

 

Nomenclature
HGNC (Hugo)NONO   7871
Cards
AtlasNONOID168
Atlas Explorer : (Salamanque)NONO
Entrez_Gene (NCBI)NONO    non-POU domain containing octamer binding
AliasesMRXS34; NMT55; NRB54; P54; 
P54NRB; PPP1R114
GeneCards (Weizmann)NONO
Ensembl hg19 (Hinxton)ENSG00000147140 [Gene_View]
Ensembl hg38 (Hinxton)ENSG00000147140 [Gene_View]  ENSG00000147140 [Sequence]  chrX:71283635-71301168 [Contig_View]  NONO [Vega]
ICGC DataPortalENSG00000147140
TCGA cBioPortalNONO
AceView (NCBI)NONO
Genatlas (Paris)NONO
SOURCE (Princeton)NONO
Genetics Home Reference (NIH)NONO
Genomic and cartography
GoldenPath hg38 (UCSC)NONO  -     chrX:71283635-71301168 +  Xq13.1   [Description]    (hg38-Dec_2013)
GoldenPath hg19 (UCSC)NONO  -     Xq13.1   [Description]    (hg19-Feb_2009)
GoldenPathNONO - Xq13.1 [CytoView hg19]  NONO - Xq13.1 [CytoView hg38]
ImmunoBaseENSG00000147140
Genome Data Viewer NCBINONO [Mapview hg19]  
OMIM300084   300967   
Gene and transcription
Genbank (Entrez)AK055406 AK291140 AK297144 AU119361 BC002364
RefSeq transcript (Entrez)NM_001145408 NM_001145409 NM_001145410 NM_007363
Consensus coding sequences : CCDS (NCBI)NONO
Gene ExpressionNONO [ NCBI-GEO ]   NONO [ EBI - ARRAY_EXPRESS ]   NONO [ SEEK ]   NONO [ MEM ]
Gene Expression Viewer (FireBrowse)NONO [ Firebrowse - Broad ]
GenevisibleExpression of NONO in : [tissues]  [cell-lines]  [cancer]  [perturbations]  
BioGPS (Tissue expression)4841
GTEX Portal (Tissue expression)NONO
Human Protein AtlasENSG00000147140-NONO [pathology]   [cell]   [tissue]
Protein : pattern, domain, 3D structure
UniProt/SwissProtQ15233   [function]  [subcellular_location]  [family_and_domains]  [pathology_and_biotech]  [ptm_processing]  [expression]  [interaction]
NextProtQ15233  [Sequence]  [Exons]  [Medical]  [Publications]
With graphics : InterProQ15233
PhosPhoSitePlusQ15233
Domaine pattern : Prosite (Expaxy)RRM (PS50102)   
Domains : Interpro (EBI)NOPS    Nucleotide-bd_a/b_plait_sf    p54nrb_RRM1    RBD_domain_sf    RRM_dom   
Domain families : Pfam (Sanger)NOPS (PF08075)    RRM_1 (PF00076)   
Domain families : Pfam (NCBI)pfam08075    pfam00076   
Domain families : Smart (EMBL)RRM (SM00360)  
Conserved Domain (NCBI)NONO
PDB (RSDB)3SDE    5IFM   
PDB Europe3SDE    5IFM   
PDB (PDBSum)3SDE    5IFM   
PDB (IMB)3SDE    5IFM   
Structural Biology KnowledgeBase3SDE    5IFM   
SCOP (Structural Classification of Proteins)3SDE    5IFM   
CATH (Classification of proteins structures)3SDE    5IFM   
SuperfamilyQ15233
AlphaFold pdb e-kbQ15233   
Human Protein Atlas [tissue]ENSG00000147140-NONO [tissue]
HPRD02098
Protein Interaction databases
DIP (DOE-UCLA)Q15233
IntAct (EBI)Q15233
BioGRIDNONO
STRING (EMBL)NONO
ZODIACNONO
Ontologies - Pathways
QuickGOQ15233
Ontology : AmiGOfibrillar center  activation of innate immune response  DNA binding  chromatin binding  RNA binding  protein binding  nucleus  nucleus  nucleus  nucleoplasm  chromosome  DNA repair  DNA recombination  regulation of transcription, DNA-templated  mRNA processing  circadian rhythm  RNA splicing  membrane  nuclear matrix  nuclear speck  paraspeckles  regulation of circadian rhythm  identical protein binding  innate immune response  negative regulation of transcription, DNA-templated  RNA polymerase II transcription regulator complex  negative regulation of oxidative stress-induced neuron intrinsic apoptotic signaling pathway  
Ontology : EGO-EBIfibrillar center  activation of innate immune response  DNA binding  chromatin binding  RNA binding  protein binding  nucleus  nucleus  nucleus  nucleoplasm  chromosome  DNA repair  DNA recombination  regulation of transcription, DNA-templated  mRNA processing  circadian rhythm  RNA splicing  membrane  nuclear matrix  nuclear speck  paraspeckles  regulation of circadian rhythm  identical protein binding  innate immune response  negative regulation of transcription, DNA-templated  RNA polymerase II transcription regulator complex  negative regulation of oxidative stress-induced neuron intrinsic apoptotic signaling pathway  
NDEx NetworkNONO
Atlas of Cancer Signalling NetworkNONO
Wikipedia pathwaysNONO
Orthology - Evolution
OrthoDB4841
GeneTree (enSembl)ENSG00000147140
Phylogenetic Trees/Animal Genes : TreeFamNONO
Homologs : HomoloGeneNONO
Homology/Alignments : Family Browser (UCSC)NONO
Gene fusions - Rearrangements
Fusion : MitelmanNONO::TFE3 [Xq13.1/Xp11.23]  
Fusion : MitelmanTFE3::NONO [Xp11.23/Xq13.1]  
Fusion : COSMICNONO [Xq13.1]  -  TFE3 [Xp11.23]  [fusion_865]  
Fusion : FusionHubABI3BP--NONO    AP2A1--NONO    ARNTL--NONO    ASPSCR1--NONO    CLASRP--NONO    COPS7B--NONO    FBLIM1--NONO    IAH1--NONO    IGHG1--NONO    ITGA7--NONO   
LYZ--NONO    MKRN2OS--NONO    NONO--ABCB1    NONO--AHCY    NONO--ARL6IP4    NONO--EXOSC9    NONO--IQCB1    NONO--NSUN2    NONO--PTRF    NONO--REV1   
NONO--SFTPB    NONO--SLC25A16    NONO--SLX4IP    NONO--TARS2    NONO--TFE3    PALLD--NONO    PRM1--NONO    SORBS1--NONO    SPTBN4--NONO    SZT2--NONO   
TFE3--NONO    ZC3HAV1--NONO    ZSCAN21--NONO   
Fusion : QuiverNONO
Polymorphisms : SNP and Copy number variants
NCBI Variation ViewerNONO [hg38]
dbSNP Single Nucleotide Polymorphism (NCBI)NONO
dbVarNONO
ClinVarNONO
MonarchNONO
1000_GenomesNONO 
Exome Variant ServerNONO
GNOMAD BrowserENSG00000147140
Varsome BrowserNONO
ACMGNONO variants
VarityQ15233
Genomic Variants (DGV)NONO [DGVbeta]
DECIPHERNONO [patients]   [syndromes]   [variants]   [genes]  
CONAN: Copy Number AnalysisNONO 
Mutations
ICGC Data PortalNONO 
TCGA Data PortalNONO 
Broad Tumor PortalNONO
OASIS PortalNONO [ Somatic mutations - Copy number]
Cancer Gene: CensusNONO 
Somatic Mutations in Cancer : COSMICNONO  [overview]  [genome browser]  [tissue]  [distribution]  
Somatic Mutations in Cancer : COSMIC3DNONO
Mutations and Diseases : HGMDNONO
LOVD (Leiden Open Variation Database)[gene] [transcripts] [variants]
BioMutaNONO
DgiDB (Drug Gene Interaction Database)NONO
DoCM (Curated mutations)NONO
CIViC (Clinical Interpretations of Variants in Cancer)NONO
OncoKBNONO
Cancer3DNONO
Impact of mutations[PolyPhen2] [Provean] [Buck Institute : MutDB] [Mutation Assessor] [Mutanalyser]
Diseases
OMIM300084    300967   
Orphanet21643    24169   
DisGeNETNONO
MedgenNONO
Genetic Testing Registry NONO
NextProtQ15233 [Medical]
GENETestsNONO
Target ValidationNONO
Huge Navigator NONO [HugePedia]
ClinGenNONO
Clinical trials, drugs, therapy
MyCancerGenomeNONO
Protein Interactions : CTDNONO
Pharm GKB GenePA31680
PharosQ15233
Clinical trialNONO
Miscellaneous
canSAR (ICR)NONO
HarmonizomeNONO
ARCHS4NONO
DataMed IndexNONO
Probes
Litterature
PubMed298 Pubmed reference(s) in Entrez
GeneRIFsGene References Into Functions (Entrez)
EVEXNONO
REVIEW articlesautomatic search in PubMed
Last year publicationsautomatic search in PubMed

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indexed on : Thu Jan 20 14:13:13 CET 2022
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jlhuret@AtlasGeneticsOncology.org.