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Gene Review

PRCC  -  papillary renal cell carcinoma...

Homo sapiens

Synonyms: Papillary renal cell carcinoma translocation-associated gene protein, Proline-rich protein PRCC, RCCP1, TPRC
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Disease relevance of PRCC

  • Fusion of the transcription factor TFE3 gene to a novel gene, PRCC, in t(X;1)(p11;q21)-positive papillary renal cell carcinomas [1].
  • PRCC-TFE3 renal carcinomas: morphologic, immunohistochemical, ultrastructural, and molecular analysis of an entity associated with the t(X;1)(p11.2;q21) [2].
  • There is no effective therapy for metastatic PRCC, and patients are often excluded from kidney cancer trials [3].
  • Furthermore, papillary adenomas were more commonly found in kidneys removed for PRCC (25%, 18/71) than in kidneys harboring clear-cell RCC (1.9%, 6/318) [4].
  • The predominant expression profiles were as follows: CRCC, CK7-/KIT-/PAX2+ (26/37); PRCC, CK7+/KIT-/PAX2+ (17/20); ChC, CK7+/KIT+/PAX2- (10/11); and oncocytoma, CK7-/KIT+/PAX2+ (19/23) [5].

Psychiatry related information on PRCC

  • It is suggested that genetic differences between PRCC and other RCC variants may be exploited in the future for surgical decision making [6].

High impact information on PRCC

  • The papillary renal cell carcinoma (RCC)-associated (X;1)(p11;q21) translocation fuses the genes PRCC and TFE3 and leads to cancer by an unknown molecular mechanism [7].
  • We here demonstrate that the mitotic checkpoint protein MAD2B interacts with PRCC [7].
  • PRCC is ubiquitously expressed in normal adult and fetal tissues and encodes a putative protein of 491 aa with a relatively high content of prolines [1].
  • We studied the gene expression profiles of 34 cases of PRCC using Affymetrix HGU133 Plus 2.0 arrays (54,675 probe sets) using both unsupervised and supervised analyses [3].
  • Alterations of known genes in PRCC include missense mutations in the MET oncogene (7q31) and rare translocations fusing TFE3 at Xp11.2 with a variety of other loci [8].

Chemical compound and disease context of PRCC


Biological context of PRCC


Anatomical context of PRCC

  • In our experience, PRCC tend to loco-regional invasion with lymph node spread [13].
  • Morphologically, PRCC differs from other forms of RCC in that it is associated with frequent tumor infiltration by macrophages and lymphocytes, and a tendency for central necrosis and cystic change [14].
  • PRCC, usually a low-grade neoplasm, may be associated with cystic degeneration, hemorrhage, and presence of abundant hemosiderin-laden macrophages (HLM) [15].
  • In untreated plasma, TPRC measured by IRMA was lower than expected, but in amniotic fluid expected values were obtained [16].

Associations of PRCC with chemical compounds

  • The translocation is predicted to result in the fusion of the N-terminal region of the PRCC protein, which includes a proline-rich domain, to the entire TFE3 protein [17].
  • Whereas ferrous AXCP reacts with NO to form a predominantly five-coordinate heme-nitrosyl complex via a six-coordinate intermediate, RCCP forms an equilibrium mixture of six-coordinate and five-coordinate heme-nitrosyl species in approximately equal proportions [18].
  • Evidence that RCCP has a more accessible distal coordination site than in AXCP stems from the fact that ferric RCCP readily forms a heme complex with exogenous imidazole, whereas AXCP does not [18].
  • Plasma angiotensin II correlated significantly with APRA, TPRA, TPRC and plasma angiotensin I (PA I), but not with inactive renin, which suggests that inactive renin does not produce angiotensin II in vivo [19].
  • During captopril and saralasin significant increases in TPRC and APRC were found and no change in IPRC [20].

Regulatory relationships of PRCC

  • RESULTS: PPET-1 was found to be up-regulated in ccRCC tumor specimens and down-regulated in PRCC tumor specimens [21].

Other interactions of PRCC

  • Impairment of MAD2B-PRCC interaction in mitotic checkpoint defective t(X;1)-positive renal cell carcinomas [7].
  • Although CCRCC and PRCC share most immunomarkers, CK7 and AMACR expression can be helpful in the differential diagnosis of challenging histologic variants of the two [22].
  • In addition, E-cadherin aids in the distinction between types 1 and 2 PRCC [22].
  • ECE-1 was expressed in all tissue specimens at comparable levels, with moderate but significant elevation in normal tissue specimens associated with PRCC [21].
  • The percent positive staining of MMP-9 at the invasive front of tumor cells was significantly higher in CRCC than in ChRCC, PRCC, or CoRCC [23].

Analytical, diagnostic and therapeutic context of PRCC


  1. Fusion of the transcription factor TFE3 gene to a novel gene, PRCC, in t(X;1)(p11;q21)-positive papillary renal cell carcinomas. Weterman, M.A., Wilbrink, M., Geurts van Kessel, A. Proc. Natl. Acad. Sci. U.S.A. (1996) [Pubmed]
  2. PRCC-TFE3 renal carcinomas: morphologic, immunohistochemical, ultrastructural, and molecular analysis of an entity associated with the t(X;1)(p11.2;q21). Argani, P., Antonescu, C.R., Couturier, J., Fournet, J.C., Sciot, R., Debiec-Rychter, M., Hutchinson, B., Reuter, V.E., Boccon-Gibod, L., Timmons, C., Hafez, N., Ladanyi, M. Am. J. Surg. Pathol. (2002) [Pubmed]
  3. A molecular classification of papillary renal cell carcinoma. Yang, X.J., Tan, M.H., Kim, H.L., Ditlev, J.A., Betten, M.W., Png, C.E., Kort, E.J., Futami, K., Furge, K.A., Takahashi, M., Kanayama, H.O., Tan, P.H., Teh, B.S., Luan, C., Wang, K., Pins, M., Tretiakova, M., Anema, J., Kahnoski, R., Nicol, T., Stadler, W., Vogelzang, N.G., Amato, R., Seligson, D., Figlin, R., Belldegrun, A., Rogers, C.G., Teh, B.T. Cancer Res. (2005) [Pubmed]
  4. Renal papillary adenoma-a putative precursor of papillary renal cell carcinoma. Wang, K.L., Weinrach, D.M., Luan, C., Han, M., Lin, F., Teh, B.T., Yang, X.J. Hum. Pathol. (2007) [Pubmed]
  5. Immunohistochemical Analysis for Cytokeratin 7, KIT, and PAX2: Value in the Differential Diagnosis of Chromophobe Cell Carcinoma. Memeo, L., Jhang, J., Assaad, A.M., McKiernan, J.M., Murty, V.V., Hibshoosh, H., Tong, G.X., Mansukhani, M.M. Am. J. Clin. Pathol. (2007) [Pubmed]
  6. The Cleveland Clinic experience with papillary (chromophil) renal cell carcinoma: clinical outcome with histopathological correlation. Chow, G.K., Myles, J., Novick, A.C. The Canadian journal of urology. (2001) [Pubmed]
  7. Impairment of MAD2B-PRCC interaction in mitotic checkpoint defective t(X;1)-positive renal cell carcinomas. Weterman, M.A., van Groningen, J.J., Tertoolen, L., van Kessel, A.G. Proc. Natl. Acad. Sci. U.S.A. (2001) [Pubmed]
  8. Unique patterns of allelic imbalance distinguish type 1 from type 2 sporadic papillary renal cell carcinoma. Sanders, M.E., Mick, R., Tomaszewski, J.E., Barr, F.G. Am. J. Pathol. (2002) [Pubmed]
  9. Novel mutation in the ATP-binding site of the MET oncogene tyrosine kinase in a HPRCC family. Olivero, M., Valente, G., Bardelli, A., Longati, P., Ferrero, N., Cracco, C., Terrone, C., Rocca-Rossetti, S., Comoglio, P.M., Di Renzo, M.F. Int. J. Cancer (1999) [Pubmed]
  10. Upregulation of the transcription factor TFEB in t(6;11)(p21;q13)-positive renal cell carcinomas due to promoter substitution. Kuiper, R.P., Schepens, M., Thijssen, J., van Asseldonk, M., van den Berg, E., Bridge, J., Schuuring, E., Schoenmakers, E.F., van Kessel, A.G. Hum. Mol. Genet. (2003) [Pubmed]
  11. PRCC, the commonest TFE3 fusion partner in papillary renal carcinoma is associated with pre-mRNA splicing factors. Skalsky, Y.M., Ajuh, P.M., Parker, C., Lamond, A.I., Goodwin, G., Cooper, C.S. Oncogene (2001) [Pubmed]
  12. Isolation and characterization of the Xenopus laevis orthologs of the human papillary renal cell carcinoma-associated genes PRCC and MAD2L2 (MAD2B). van den Hurk, W.H., Martens, G.J., Geurts van Kessel, A., van Groningen, J.J. Cytogenet. Genome Res. (2004) [Pubmed]
  13. A comparative study of metastatic renal cell carcinoma with correlation to subtype and primary tumor. Mai, K.T., Landry, D.C., Robertson, S.J., Commons, A.S., Burns, B.F., Thijssen, A., Collins, J. Pathol. Res. Pract. (2001) [Pubmed]
  14. DNA ploidy pattern in papillary renal cell carcinoma. Correlation with clinicopathological parameters and survival. del Vecchio, M.T., Lazzi, S., Bruni, A., Mangiavacchi, P., Cevenini, G., Luzi, P. Pathol. Res. Pract. (1998) [Pubmed]
  15. Abundant intracytoplasmic hemosiderin in both histiocytes and neoplastic cells: A diagnostic pitfall in fine-needle aspiration of cystic papillary renal-cell carcinoma. Wang, S., Filipowicz, E.A., Schnadig, V.J. Diagn. Cytopathol. (2001) [Pubmed]
  16. A two-site monoclonal immunoradiometric assay for total renin protein: comparison with an established enzyme kinetic assay. Thatcher, R.L., Whitworth, J.A., Casley, D.G., Johnston, C.I., Skinner, S.L. Clin. Exp. Pharmacol. Physiol. (1988) [Pubmed]
  17. 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, S.K., Clark, J., Gill, S., Hamoudi, R., Crew, A.J., Gwilliam, R., Ross, M., Linehan, W.M., Birdsall, S., Shipley, J., Cooper, C.S. Hum. Mol. Genet. (1996) [Pubmed]
  18. Accessibility of the distal heme face, rather than Fe-His bond strength, determines the heme-nitrosyl coordination number of cytochromes c': evidence from spectroscopic studies. Andrew, C.R., Kemper, L.J., Busche, T.L., Tiwari, A.M., Kecskes, M.C., Stafford, J.M., Croft, L.C., Lu, S., Moënne-Loccoz, P., Huston, W., Moir, J.W., Eady, R.R. Biochemistry (2005) [Pubmed]
  19. Active and inactive renin in normal human plasma. Comparison between acid activation and cryoactivation. Lijnen, P.J., Amery, A.K., Fagard, R.H. Clin. Chim. Acta (1979) [Pubmed]
  20. Biological significance of active and inactive renin in hypertensive patients. Lijnen, P., Fagard, R., Staessen, J., Amery, A. Clinical and experimental hypertension. Part A, Theory and practice. (1982) [Pubmed]
  21. Endothelin axis expression is markedly different in the two main subtypes of renal cell carcinoma. Douglas, M.L., Richardson, M.M., Nicol, D.L. Cancer (2004) [Pubmed]
  22. The usefulness of immunohistochemical markers in the differential diagnosis of renal neoplasms. Zhou, M., Roma, A., Magi-Galluzzi, C. Clin. Lab. Med. (2005) [Pubmed]
  23. Increasing expression of extracellular matrix metalloprotease inducer in renal cell carcinoma: tissue microarray analysis of immunostaining score with clinicopathological parameters. Jin, J.S., Hsieh, D.S., Lin, Y.F., Wang, J.Y., Sheu, L.F., Lee, W.H. International journal of urology : official journal of the Japanese Urological Association. (2006) [Pubmed]
  24. Malignant papillary renal tumors with extensive clear cell change: a molecular analysis by microsatellite analysis and fluorescence in situ hybridization. Salama, M.E., Worsham, M.J., DePeralta-Venturina, M. Arch. Pathol. Lab. Med. (2003) [Pubmed]
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