PMID- 10644981
OWN - NLM
STAT- MEDLINE
DCOM- 20000207
LR  - 20061115
IS  - 0950-9232 (Print)
IS  - 0950-9232 (Linking)
VI  - 19
IP  - 1
DP  - 2000 Jan 6
TI  - Nuclear localization and transactivating capacities of the papillary renal cell
      carcinoma-associated TFE3 and PRCC (fusion) proteins.
PG  - 69-74
AB  - The papillary renal cell carcinoma-associated t(X;1)(p11;q21) leads to fusion of 
      the transcription factor TFE3 gene on the X-chromosome to a novel gene, PRCC, on 
      chromosome 1. As a result, two putative fusion proteins are formed: PRCCTFE3,
      which contains all known domains for DNA binding, dimerization, and
      transactivation of the TFE3 protein, and the reciprocal product TFE3PRCC. Upon
      transfection into COS cells, both wild type and fusion proteins were found to be 
      located in the nucleus. When comparing the transactivating capacities of these
      (fusion) proteins, significant differences were noted. PRCCTFE3 acted as a
      threefold better transactivator than wild type TFE3 both in a TFE3-specific and
      in a general (Zebra) reporter assay. In addition, PRCC and the two fusion
      proteins were found to be potent transactivators in the Zebra reporter assay. We 
      propose that, as a result of the (X;1) translocation, fusion of the N-terminal
      PRCC sequences to TFE3 alters the transactivation capacity of the transcription
      factor thus leading to aberrant gene regulation and, ultimately, tumor formation.
FAU - Weterman, M J
AU  - Weterman MJ
AD  - Department of Human Genetics 417, University Hospital Nijmegen, 6500 HB Nijmegen,
      The Netherlands.
FAU - van Groningen, J J
AU  - van Groningen JJ
FAU - Jansen, A
AU  - Jansen A
FAU - van Kessel, A G
AU  - van Kessel AG
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - England
TA  - Oncogene
JT  - Oncogene
JID - 8711562
RN  - 0 (Basic Helix-Loop-Helix Leucine Zipper Transcription Factors)
RN  - 0 (Cell Cycle Proteins)
RN  - 0 (DNA-Binding Proteins)
RN  - 0 (Fv1 protein, mouse)
RN  - 0 (Neoplasm Proteins)
RN  - 0 (Proteins)
RN  - 0 (Recombinant Fusion Proteins)
RN  - 0 (TFE3 protein, human)
RN  - 0 (Trans-Activators)
RN  - 0 (Transcription Factors)
SB  - IM
MH  - Animals
MH  - Basic Helix-Loop-Helix Leucine Zipper Transcription Factors
MH  - COS Cells
MH  - Carcinoma, Renal Cell/*genetics
MH  - *Cell Cycle Proteins
MH  - Cell Nucleus/*chemistry
MH  - DNA-Binding Proteins/*analysis/physiology
MH  - Kidney Medulla
MH  - Kidney Neoplasms/*genetics
MH  - *Neoplasm Proteins
MH  - Proteins/*analysis/physiology
MH  - Recombinant Fusion Proteins/*analysis/physiology
MH  - Trans-Activators/*analysis/physiology
MH  - Transcription Factors/*analysis/physiology
EDAT- 2000/01/25 00:00
MHDA- 2000/01/25 00:01
CRDT- 2000/01/25 00:00
PHST- 2000/01/25 00:00 [pubmed]
PHST- 2000/01/25 00:01 [medline]
PHST- 2000/01/25 00:00 [entrez]
AID - 10.1038/sj.onc.1203255 [doi]
PST - ppublish
SO  - Oncogene. 2000 Jan 6;19(1):69-74. doi: 10.1038/sj.onc.1203255.