Skip Navigation

This Article
Right arrow Full Text Freely available
Right arrow FREE Full Text (PDF) Freely available
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in ISI Web of Science
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrow Search for citing articles in:
ISI Web of Science (102)
Right arrowRequest Permissions
Google Scholar
Right arrow Articles by Sidhar, S. K.
Right arrow Articles by Cooper, C. S.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Sidhar, S. K.
Right arrow Articles by Cooper, C. S.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

Human Molecular Genetics, Vol 5, 1333-1338, Copyright © 1996 by Oxford University Press

ARTICLES

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

SK Sidhar, J Clark, S Gill, R Hamoudi, AJ Crew, R Gwilliam, M Ross, WM Linehan, S Birdsall, J Shipley and CS Cooper
Molecular Carcinogenesis Section, Institute of Cancer Research, Haddow Laboratories, Belmont, UK.

The specific chromosomal translocation t(X;1)(p11.2;q21.2) has been observed in human papillary renal cell carcinomas. In this study we demonstrated that this translocation results in the fusion of a novel gene designated PRCC at 1q21.2 to the TFE3 gene at Xp11.2. TFE3 encodes a member of the basic helix-loop-helix (bHLH) family of transcription factors originally identified by its ability to bind to microE3 elements in the immunoglobin heavy chain intronic enhancer. 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. Notably the generation of the chimaeric PRCC- TFE3 gene appears to be accompanied by complete loss of normal TFE3 transcripts. This work establishes that the disruption of transcriptional control by chromosomal translocation is important in the development of kidney carcinoma in addition to its previously established role in the aetiology of sarcomas and leukaemias.
Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?


This article has been cited by other articles:


Home page
 INT J SURG PATHOLHome page
A. Righi, K. Dimosthenous, and J. Rosai
PEComa: Another Member of the MiT Tumor Family?
International Journal of Surgical Pathology, January 1, 2008; 16(1): 16 - 20.
[Abstract] [PDF]

Home page
 Cancer Res.Home page
M. Tsuda, I. J. Davis, P. Argani, N. Shukla, G. G. McGill, M. Nagai, T. Saito, M. Lae, D. E. Fisher, and M. Ladanyi
TFE3 Fusions Activate MET Signaling by Transcriptional Up-regulation, Defining Another Class of Tumors as Candidates for Therapeutic MET Inhibition
Cancer Res., February 1, 2007; 67(3): 919 - 929.
[Abstract] [Full Text] [PDF]

Home page
 Genes Dev.Home page
L. Chin, L. A. Garraway, and D. E. Fisher
Malignant melanoma: genetics and therapeutics in the genomic era.
Genes & Dev., August 15, 2006; 20(16): 2149 - 2182.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
S. M. B. Nijman, E. M. Hijmans, S. E. Messaoudi, M. M. W. van Dongen, C. Sardet, and R. Bernards
A Functional Genetic Screen Identifies TFE3 as a Gene That Confers Resistance to the Anti-proliferative Effects of the Retinoblastoma Protein and Transforming Growth Factor-beta
J. Biol. Chem., August 4, 2006; 281(31): 21582 - 21587.
[Abstract] [Full Text] [PDF]

Home page
 NEJMHome page
H. T. Cohen and F. J. McGovern
Renal-Cell Carcinoma
N. Engl. J. Med., December 8, 2005; 353(23): 2477 - 2490.
[Full Text] [PDF]

Home page
 Mol Cancer ResHome page
H. Qi, M. L. Gervais, W. Li, J. A. DeCaprio, J. R.G. Challis, and M. Ohh
Molecular Cloning and Characterization of the von Hippel-Lindau-Like Protein
Mol. Cancer Res., January 1, 2004; 2(1): 43 - 52.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
R. P. Kuiper, M. Schepens, J. Thijssen, M. van Asseldonk, E. van den Berg, J. Bridge, E. Schuuring, E. F.P.M. Schoenmakers, and A. G. van Kessel
Upregulation of the transcription factor TFEB in t(6;11)(p21;q13)-positive renal cell carcinomas due to promoter substitution
Hum. Mol. Genet., July 15, 2003; 12(14): 1661 - 1669.
[Abstract] [Full Text] [PDF]

Home page
 Proc. Natl. Acad. Sci. USAHome page
I. J. Davis, B.-L. Hsi, J. D. Arroyo, S. O. Vargas, Y. A. Yeh, G. Motyckova, P. Valencia, A. R. Perez-Atayde, P. Argani, M. Ladanyi, et al.
Cloning of an Alpha-TFEB fusion in renal tumors harboring the t(6;11)(p21;q13) chromosome translocation
PNAS, May 13, 2003; 100(10): 6051 - 6056.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
D. Bodmer, W. van den Hurk, J. J. M. van Groningen, M. J. Eleveld, G. J. M. Martens, M. A. J. Weterman, and A. Geurts van Kessel
Understanding familial and non-familial renal cell cancer
Hum. Mol. Genet., October 1, 2002; 11(20): 2489 - 2498.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Pathol.Home page
M. E. Sanders, R. Mick, J. E. Tomaszewski, and F. G. Barr
Unique Patterns of Allelic Imbalance Distinguish Type 1 from Type 2 Sporadic Papillary Renal Cell Carcinoma
Am. J. Pathol., September 1, 2002; 161(3): 997 - 1005.
[Abstract] [Full Text] [PDF]

Home page
 Proc. Natl. Acad. Sci. USAHome page
M. A. J. Weterman, J. J. M. van Groningen, L. Tertoolen, and A. G. van Kessel
Impairment of MAD2B-PRCC interaction in mitotic checkpoint defective t(X;1)-positive renal cell carcinomas
PNAS, November 20, 2001; 98(24): 13808 - 13813.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Pathol.Home page
P. Argani, C. R. Antonescu, P. B. Illei, M. Y. Lui, C. F. Timmons, R. Newbury, V. E. Reuter, A. J. Garvin, A. R. Perez-Atayde, J. A. Fletcher, et al.
Primary Renal Neoplasms with the ASPL-TFE3 Gene Fusion of Alveolar Soft Part Sarcoma : A Distinctive Tumor Entity Previously Included among Renal Cell Carcinomas of Children and Adolescents
Am. J. Pathol., July 1, 2001; 159(1): 179 - 192.
[Abstract] [Full Text] [PDF]

Home page
 Cancer Res.Home page
P. Heimann, H. El Housni, G. Ogur, M. A. J. Weterman, E. M. Petty, and G. Vassart
Fusion of a Novel Gene, RCC17, to the TFE3 gene in t(X;17)(p11.2;q25.3)-bearing Papillary Renal Cell Carcinomas
Cancer Res., May 1, 2001; 61(10): 4130 - 4135.
[Abstract] [Full Text]

Home page
 J. Clin. Endocrinol. Metab.Home page
C. J. Haven, F. K. Wong, E. W. C. M. van Dam, R. van der Luijt, C. van Asperen, J. Jansen, C. Rosenberg, M. de Wit, J. Roijers, J. Hoppener, et al.
A Genotypic and Histopathological Study of a Large Dutch Kindred with Hyperparathyroidism-Jaw Tumor Syndrome
J. Clin. Endocrinol. Metab., April 1, 2000; 85(4): 1449 - 1454.
[Abstract] [Full Text]

Home page
 Am. J. Pathol.Home page
I. A. Lubensky, L. Schmidt, Z. Zhuang, G. Weirich, S. Pack, N. Zambrano, M. M. Walther, P. Choyke, W. M. Linehan, and B. Zbar
Hereditary and Sporadic Papillary Renal Carcinomas with c-met Mutations Share a Distinct Morphological Phenotype
Am. J. Pathol., August 1, 1999; 155(2): 517 - 526.
[Abstract] [Full Text] [PDF]

Home page
 Proc. Natl. Acad. Sci. USAHome page
M. A. J. Weterman, M. Wilbrink, and A. G. van Kessel
Fusion of the transcription factor TFE3 gene to a novel gene, PRCC, in t(X;1)(p11;q21)-positive papillary renal cell carcinomas
PNAS, December 24, 1996; 93(26): 15294 - 15298.
[Abstract] [Full Text] [PDF]


Disclaimer: Please note that abstracts for content published before 1996 were created through digital scanning and may therefore not exactly replicate the text of the original print issues. All efforts have been made to ensure accuracy, but the Publisher will not be held responsible for any remaining inaccuracies. If you require any further clarification, please contact our Customer Services Department.