Written | 1999-01 | François Desangles, Jean-Loup Huret |
Laboratoire de Biologie, Hopital du Val de Grace, 75230 Paris, France |
Identity |
Alias (NCBI) | RCCP1 (renal cell carcinoma, papillary, 1) |
HGNC (Hugo) | PRCC |
HGNC Alias symb | RCCP1 |
HGNC Previous name | papillary 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] |
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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 |
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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 ] |
Other Solid tumors implicated (Data extracted from papers in the Atlas) [ 6 ] |
External links |
REVIEW articles | automatic search in PubMed |
Last year publications | automatic search in PubMed |
© Atlas of Genetics and Cytogenetics in Oncology and Haematology | indexed on : Fri Feb 19 17:58:05 CET 2021 |
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