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RNASET2 (ribonuclease T2)


Other namesRNASE6PL
LocusID (NCBI) 8635
Location 6q27
Location_base_pair Starts at 167343004 and ends at 167370077 bp from pter ( according to hg19-Feb_2009)  [Mapping]
Local_order Telomeric to RPS6KA2, centromeric to FGFR1OP.
Note This gene is the first human member of the Rh/T2/S-glycoprotein family of extracellular ribonucleases. It is a putative class II tumor suppressor gene potentially involved in the pathogenesis of several solid and haematologic human neoplasias such as ovarian cancer, melanoma and non-Hodgkin lymphoma.


  I-IX: RNASET2 exons.
Description This gene is split in 9 exons spanning approximatly 27 kb of genomic DNA. The translation initiation codon is located to exon 1 and the stop codon to exon 9. Exons III and VI encode the two CAS motifs (Catalytic Active Sites) responsible for the ribonuclease activity of the RNASET2 protein.
Transcription The RNASET2 gene is transcribed in the telomere-to-centromere orientation to produce an ubiquitously expressed mRNA approximately 1,4 kb in length. EST clones representing splice variants of the same gene have been described.
Pseudogene A processed pseudogene showing 85% identity with RNASET2 mRNA maps to chromosome 7p11.2. The expression pattern of this pseudogene is not known.


Description The full-length RNASET2 protein contains 256 aminoacids and displays an apparent MW of 36 kDa in its secreted form. Two 31 and 27 kDa C-terminal proteolytic products have also been observed intracellularly in several human cancer cell lines and localize to the lysosome.
Expression Expression of the RNASET2 protein has been detected in several human ovarian cancer cell lines and in some melanoma, prostate, pancreatic and breast carcinoma cell lines.
Localisation The RNASET2 protein can be detected either intracellularly within lysosomes and secretory pathway, or extracellularly in a secreted form (in cell culture supernatants).
Function Biochemical function : RNASET2 is an acid ribonuclease with optimal activity at pH 5 and preferential cleavage of poly-A and poly-U homo-polyribonucleotides.
Biological function : RNASET2 behaves as a class II tumor suppressor gene for ovarian cancer, since experimental overexpression of this gene in human ovarian cancer cell lines is associated with a significant decrease of their tumorigenic and metastasizing potential in vivo. Strikingly, the ribonuclease catalytic activity is apparently dispensable for RNASET2 to play such antioncogenic role. Indeed, a double CAS mutant cDNA construct encoding an almost inactive RNASET2 protein is still able to suppress tumorigenicity and metastasis when overexpressed in the same ovarian cancer cell lines. Such results have been recently confirmed in a human melanoma cell line.
Homology The primary sequenze of RNASET2 shows strong homology to the Rh/T2/S family of secreted ribonucleases.


Germinal A common exon-9 missense C708T germline mutation has been described but no evidence for an association of this allele with human cancer was found.
Somatic A few common polymorphisms in exons 1, 8 and 9 have been described.

Implicated in

Entity Human ovarian carcinoma.
Disease Loss of expression of RNASET2 occurs in a significant fraction of human ovarian cancer cell lines and primary ovarian tumours. When overexpressed by gene transfer experiments in human ovarian cancer cell lines displaying a low level of endogenous mRNA, RNASET2 strongly suppresses the tumorigenic and metastatic potential of these cell lines in vivo.
Cytogenetics The RNASET2 genes maps in a genomic region (6q27) which is frequently deleted or otherwise rearranged in a wide range of human neoplasias, including ovarian cancer.

External links

HGNC (Hugo)RNASET2   21686
Entrez_Gene (NCBI)RNASET2  8635  ribonuclease T2
GeneCards (Weizmann)RNASET2
Ensembl (Hinxton)ENSG00000026297 [Gene_View]  chr6:167343004-167370077 [Contig_View]  RNASET2 [Vega]
ICGC DataPortalENSG00000026297
Genatlas (Paris)RNASET2
SOURCE (Princeton)NM_003730
Genomic and cartography
GoldenPath (UCSC)RNASET2  -  6q27   chr6:167343004-167370077 -  6q27   [Description]    (hg19-Feb_2009)
EnsemblRNASET2 - 6q27 [CytoView]
Mapping of homologs : NCBIRNASET2 [Mapview]
OMIM612944   612951   
Gene and transcription
Genbank (Entrez)AJ419865 AJ419866 AJ419867 AK001769 AK124363
RefSeq transcript (Entrez)NM_003730
RefSeq genomic (Entrez)AC_000138 NC_000006 NC_018917 NG_016280 NT_025741 NW_001838993 NW_004929328
Consensus coding sequences : CCDS (NCBI)RNASET2
Cluster EST : UnigeneHs.529989 [ NCBI ]
CGAP (NCI)Hs.529989
Alternative Splicing : Fast-db (Paris)GSHG0027066
Alternative Splicing GalleryENSG00000026297
Gene ExpressionRNASET2 [ NCBI-GEO ]     RNASET2 [ SEEK ]   RNASET2 [ MEM ]
Protein : pattern, domain, 3D structure
UniProt/SwissProtO00584 (Uniprot)
NextProtO00584  [Medical]
With graphics : InterProO00584
Splice isoforms : SwissVarO00584 (Swissvar)
Catalytic activity : Enzyme3.1.27.- [ Enzyme-Expasy ]   3.1.27.-3.1.27.- [ IntEnz-EBI ]   3.1.27.- [ BRENDA ]   3.1.27.- [ KEGG ]   
Domaine pattern : Prosite (Expaxy)RNASE_T2_1 (PS00530)    RNASE_T2_2 (PS00531)   
Domains : Interpro (EBI)RNase_T2-like    RNase_T2_AS   
Related proteins : CluSTrO00584
Domain families : Pfam (Sanger)Ribonuclease_T2 (PF00445)   
Domain families : Pfam (NCBI)pfam00445   
DMDM Disease mutations8635
Blocks (Seattle)O00584
PDB (SRS)3T0O   
PDB (PDBSum)3T0O   
PDB (IMB)3T0O   
Human Protein AtlasENSG00000026297
Peptide AtlasO00584
IPIIPI00414896   IPI00152444   IPI00939604   IPI00975923   IPI00152442   IPI00967269   IPI00964721   
Protein Interaction databases
IntAct (EBI)O00584
Interologous Interaction database O00584
Ontologies - Pathways
Ontology : AmiGORNA binding  ribonuclease activity  extracellular region  extracellular space  lysosome  endoplasmic reticulum lumen  RNA catabolic process  ribonuclease T2 activity  lysosomal lumen  extracellular vesicular exosome  RNA phosphodiester bond hydrolysis  RNA phosphodiester bond hydrolysis  RNA phosphodiester bond hydrolysis, endonucleolytic  
Ontology : EGO-EBIRNA binding  ribonuclease activity  extracellular region  extracellular space  lysosome  endoplasmic reticulum lumen  RNA catabolic process  ribonuclease T2 activity  lysosomal lumen  extracellular vesicular exosome  RNA phosphodiester bond hydrolysis  RNA phosphodiester bond hydrolysis  RNA phosphodiester bond hydrolysis, endonucleolytic  
Protein Interaction DatabaseRNASET2
Wikipedia pathwaysRNASET2
Gene fusion - rearrangments
Polymorphisms : SNP, mutations, diseases
SNP Single Nucleotide Polymorphism (NCBI)RNASET2
Genetic variants : HAPMAPRNASET2
ICGC programENSG00000026297 
CONAN: Copy Number AnalysisRNASET2 
Somatic Mutations in Cancer : COSMICRNASET2 
LOVD (Leiden Open Variation Database)Whole genome datasets
LOVD (Leiden Open Variation Database)LOVD - Leiden Open Variation Database
LOVD (Leiden Open Variation Database)LOVD 3.0 shared installation
Mutations and Diseases : HGMDRNASET2
OMIM612944    612951   
Disease Genetic AssociationRNASET2
Huge Navigator RNASET2 [HugePedia]  RNASET2 [HugeCancerGEM]
Genomic VariantsRNASET2  RNASET2 [DGVbeta]
Exome VariantRNASET2
snp3D : Map Gene to Disease8635
General knowledge
Homologs : HomoloGeneRNASET2
Homology/Alignments : Family Browser (UCSC)RNASET2
Phylogenetic Trees/Animal Genes : TreeFamRNASET2
Chemical/Protein Interactions : CTD8635
Chemical/Pharm GKB GenePA128394541
Clinical trialRNASET2
Cancer Resource (Charite)ENSG00000026297
Other databases
PubMed35 Pubmed reference(s) in Entrez


Mammalian Rh/T2/S-glycoprotein ribonuclease family genes: cloning of a human member located in a region of chromosome 6 (6q27) frequently deleted in human malignancies.
Trubia M, Sessa L, Taramelli R
Genomics. 1997 ; 42 (2) : 342-344.
PMID 9192857
Cloning and characterization of a senescence inducing and class II tumor suppressor gene in ovarian carcinoma at chromosome region 6q27.
Acquati F, Morelli C, Cinquetti R, Bianchi MG, Porrini D, Varesco L, Gismondi V, Rocchetti R, Talevi S, Possati L, Magnanini C, Tibiletti MG, Bernasconi B, Daidone MG, Shridhar V, Smith DI, Negrini M, Barbanti-Brodano G, Taramelli R
Oncogene. 2001 ; 20 (8) : 980-988.
PMID 11314033
Molecular cloning, tissue distribution, and chromosomal localization of the human homolog of the R2/Th/Stylar ribonuclease gene family.
Acquati F, Nucci C, Bianchi MG, Gorletta T, Taramelli R
Methods in molecular biology (Clifton, N.J.). 2001 ; 160 : 87-101.
PMID 11265308
Tumor and metastasis suppression by the human RNASET2 gene.
Acquati F, Possati L, Ferrante L, Campomenosi P, Talevi S, Bardelli S, Margiotta C, Russo A, Bortoletto E, Rocchetti R, Calza R, Cinquetti R, Monti L, Salis S, Barbanti-Brodano G, Taramelli R
International journal of oncology. 2005 ; 26 (5) : 1159-1168.
PMID 15809705
Characterization of RNASET2, the first human member of the Rh/T2/S family of glycoproteins.
Campomenosi P, Salis S, Lindqvist C, Mariani D, Nordstrˆm T, Acquati F, Taramelli R
Archives of biochemistry and biophysics. 2006 ; 449 (1-2) : 17-26.
PMID 16620762
REVIEW articlesautomatic search in PubMed
Last year publicationsautomatic search in PubMed

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Written02-2007Francesco Acquati, Paola Campomenosi


This paper should be referenced as such :
Campomenosi, P ; Acquati, F
RNASET2 (ribonuclease T2)
Atlas Genet Cytogenet Oncol Haematol. 2007;11(3):219-221.
Free online version   Free pdf version   [Bibliographic record ]

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indexed on : Mon Oct 13 13:30:50 CEST 2014

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