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PRCC (papillary renal cell carcinoma)

Written1999-01François Desangles, Jean-Loup Huret
Laboratoire de Biologie, Hopital du Val de Grace, 75230 Paris, France

(Note : for Links provided by Atlas : click)

Identity

Alias (NCBI)RCCP1 (renal cell carcinoma, papillary, 1)
HGNC (Hugo) PRCC
HGNC Alias symbRCCP1
HGNC Previous namepapillary renal cell carcinoma (translocation-associated)
LocusID (NCBI) 5546
Atlas_Id 69
Location 1q23.1  [Link to chromosome band 1q23]
Location_base_pair Starts at 156767535 and ends at 156800814 bp from pter ( according to GRCh38/hg38-Dec_2013)  [Mapping PRCC.png]
 
  Probe(s) - Courtesy Mariano Rocchi, Resources for Molecular Cytogenetics
Fusion genes
(updated 2017)
Data from Atlas, Mitelman, Cosmic Fusion, Fusion Cancer, TCGA fusion databases with official HUGO symbols (see references in chromosomal bands)
ARHGAP6 (Xp22.2)::PRCC (1q23.1)EFHC1 (6p12.2)::PRCC (1q23.1)MAD2L2 (1p36.22)::PRCC (1q23.1)
PRCC (1q23.1)::HAPLN2 (1q23.1)PRCC (1q23.1)::TFE3 (Xp11.23)PRCC (1q23.1)::TMED5 (1p22.1)
SF3B2 (11q13.1)::PRCC (1q23.1)TFE3 (Xp11.23)::PRCC (1q23.1)YY1AP1 (1q22)::PRCC (1q23.1)

DNA/RNA

Description cDNA of 2039 bp
Transcription 1989 bp RNA; coding sequence: CDS 191...1666

Protein

Description 491 amino acids; 52 kDa; proline/leucine/glycine rich domain of 150 amino acids in N-term
Expression wide; in the fetus and in the adult
Homology none is known

Implicated in

Note
  
Entity t(X;1)(p11.2;q21.2) in renal cell carcinoma --> PRCC /< CC: TXT: TFE3 ID: 86 >
Disease t(X;1) is a very rare subtype of papillary renal cell carcinoma
Prognosis overall 5-yr survival rate around 85%
Hybrid/Mutated Gene 5' PRCC- 3' TFE3; variable breakpoint in PRCC; breakpoint in the 1st intron of TFE3
 
Abnormal Protein N-term PRCC with the proline rich sequence fused to most of TFE3, including the ; the reciprocal TFE3-PRCC is expressed; it is to be noted that the normal TFE3 transcript is lost in female patients.
  

Bibliography

The t(X;1)(p11.2;q21.2) translocation in papillary renal cell carcinoma fuses a novel gene PRCC to the TFE3 transcription factor gene.
Sidhar SK, Clark J, Gill S, Hamoudi R, Crew AJ, Gwilliam R, Ross M, Linehan WM, Birdsall S, Shipley J, Cooper CS
Human molecular genetics. 1996 ; 5 (9) : 1333-1338.
PMID 8872474
 
Fusion of the transcription factor TFE3 gene to a novel gene, PRCC, in t(X;1)(p11;q21)-positive papillary renal cell carcinomas.
Weterman MA, Wilbrink M, Geurts van Kessel A
Proceedings of the National Academy of Sciences of the United States of America. 1996 ; 93 (26) : 15294-15298.
PMID 8986805
 

Citation

This paper should be referenced as such :
Desangles, F ; Huret, JL
PRCC (papillary renal cell carcinoma)
Atlas Genet Cytogenet Oncol Haematol. 1999;3(2):56-56.
Free journal version : [ pdf ]   [ DOI ]


External links

 

Nomenclature
HGNC (Hugo)PRCC   9343
Cards
AtlasPRCCID69
Entrez_Gene (NCBI)PRCC    proline rich mitotic checkpoint control factor
AliasesRCCP1; TPRC
GeneCards (Weizmann)PRCC
Ensembl hg19 (Hinxton)ENSG00000143294 [Gene_View]
Ensembl hg38 (Hinxton)ENSG00000143294 [Gene_View]  ENSG00000143294 [Sequence]  chr1:156767535-156800814 [Contig_View]  PRCC [Vega]
ICGC DataPortalENSG00000143294
TCGA cBioPortalPRCC
AceView (NCBI)PRCC
Genatlas (Paris)PRCC
SOURCE (Princeton)PRCC
Genetics Home Reference (NIH)PRCC
Genomic and cartography
GoldenPath hg38 (UCSC)PRCC  -     chr1:156767535-156800814 +  1q23.1   [Description]    (hg38-Dec_2013)
GoldenPath hg19 (UCSC)PRCC  -     1q23.1   [Description]    (hg19-Feb_2009)
GoldenPathPRCC - 1q23.1 [CytoView hg19]  PRCC - 1q23.1 [CytoView hg38]
ImmunoBaseENSG00000143294
Genome Data Viewer NCBIPRCC [Mapview hg19]  
OMIM179755   605074   
Gene and transcription
Genbank (Entrez)AA846273 AK126403 AK289872 BC004913 BC010450
RefSeq transcript (Entrez)NM_005973 NM_199416
Consensus coding sequences : CCDS (NCBI)PRCC
Gene ExpressionPRCC [ NCBI-GEO ]   PRCC [ EBI - ARRAY_EXPRESS ]   PRCC [ SEEK ]   PRCC [ MEM ]
Gene Expression Viewer (FireBrowse)PRCC [ Firebrowse - Broad ]
GenevisibleExpression of PRCC in : [tissues]  [cell-lines]  [cancer]  [perturbations]  
BioGPS (Tissue expression)5546
GTEX Portal (Tissue expression)PRCC
Human Protein AtlasENSG00000143294-PRCC [pathology]   [cell]   [tissue]
Protein : pattern, domain, 3D structure
UniProt/SwissProtQ92733   [function]  [subcellular_location]  [family_and_domains]  [pathology_and_biotech]  [ptm_processing]  [expression]  [interaction]
NextProtQ92733  [Sequence]  [Exons]  [Medical]  [Publications]
With graphics : InterProQ92733
PhosPhoSitePlusQ92733
Domains : Interpro (EBI)PRCC   
Domain families : Pfam (Sanger)PRCC (PF10253)   
Domain families : Pfam (NCBI)pfam10253   
Conserved Domain (NCBI)PRCC
SuperfamilyQ92733
AlphaFold pdb e-kbQ92733   
Human Protein Atlas [tissue]ENSG00000143294-PRCC [tissue]
HPRD01562
Protein Interaction databases
DIP (DOE-UCLA)Q92733
IntAct (EBI)Q92733
BioGRIDPRCC
STRING (EMBL)PRCC
ZODIACPRCC
Ontologies - Pathways
QuickGOQ92733
Ontology : AmiGOmRNA splicing, via spliceosome  protein binding  nucleus  nucleus  nucleoplasm  cell cycle  mitotic cell cycle checkpoint signaling  nuclear speck  regulation of cell cycle  
Ontology : EGO-EBImRNA splicing, via spliceosome  protein binding  nucleus  nucleus  nucleoplasm  cell cycle  mitotic cell cycle checkpoint signaling  nuclear speck  regulation of cell cycle  
Pathways : KEGGTranscriptional misregulation in cancer   
REACTOMEQ92733 [protein]
REACTOME PathwaysR-HSA-72163 [pathway]   
NDEx NetworkPRCC
Atlas of Cancer Signalling NetworkPRCC
Wikipedia pathwaysPRCC
Orthology - Evolution
OrthoDB5546
GeneTree (enSembl)ENSG00000143294
Phylogenetic Trees/Animal Genes : TreeFamPRCC
Homologs : HomoloGenePRCC
Homology/Alignments : Family Browser (UCSC)PRCC
Gene fusions - Rearrangements
Fusion : MitelmanPRCC::HAPLN2 [1q23.1/1q23.1]  
Fusion : MitelmanPRCC::TFE3 [1q23.1/Xp11.23]  
Fusion : MitelmanPRCC::TMED5 [1q23.1/1p22.1]  
Fusion : COSMICTFE3 [Xp11.23]  -  PRCC [1q23.1]  [fusion_839]  
Fusion : QuiverPRCC
Polymorphisms : SNP and Copy number variants
NCBI Variation ViewerPRCC [hg38]
dbSNP Single Nucleotide Polymorphism (NCBI)PRCC
dbVarPRCC
ClinVarPRCC
MonarchPRCC
1000_GenomesPRCC 
Exome Variant ServerPRCC
GNOMAD BrowserENSG00000143294
Varsome BrowserPRCC
ACMGPRCC variants
VarityQ92733
Genomic Variants (DGV)PRCC [DGVbeta]
DECIPHERPRCC [patients]   [syndromes]   [variants]   [genes]  
CONAN: Copy Number AnalysisPRCC 
Mutations
ICGC Data PortalPRCC 
TCGA Data PortalPRCC 
Broad Tumor PortalPRCC
OASIS PortalPRCC [ Somatic mutations - Copy number]
Cancer Gene: CensusPRCC 
Somatic Mutations in Cancer : COSMICPRCC  [overview]  [genome browser]  [tissue]  [distribution]  
Somatic Mutations in Cancer : COSMIC3DPRCC
Mutations and Diseases : HGMDPRCC
LOVD (Leiden Open Variation Database)[gene] [transcripts] [variants]
BioMutaPRCC
DgiDB (Drug Gene Interaction Database)PRCC
DoCM (Curated mutations)PRCC
CIViC (Clinical Interpretations of Variants in Cancer)PRCC
OncoKBPRCC
NCG (London)PRCC
Cancer3DPRCC
Impact of mutations[PolyPhen2] [Provean] [Buck Institute : MutDB] [Mutation Assessor] [Mutanalyser]
Diseases
OMIM179755    605074   
Orphanet21643   
DisGeNETPRCC
MedgenPRCC
Genetic Testing Registry PRCC
NextProtQ92733 [Medical]
GENETestsPRCC
Target ValidationPRCC
Huge Navigator PRCC [HugePedia]
ClinGenPRCC
Clinical trials, drugs, therapy
MyCancerGenomePRCC
Protein Interactions : CTDPRCC
Pharm GKB GenePA33704
PharosQ92733
Clinical trialPRCC
Miscellaneous
canSAR (ICR)PRCC
HarmonizomePRCC
DataMed IndexPRCC
Probes
Litterature
PubMed40 Pubmed reference(s) in Entrez
GeneRIFsGene References Into Functions (Entrez)
EVEXPRCC
REVIEW articlesautomatic search in PubMed
Last year publicationsautomatic search in PubMed

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indexed on : Fri Oct 8 21:25:43 CEST 2021

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