PMID- 17620295
OWN - NLM
STAT- MEDLINE
DCOM- 20071025
LR  - 20130618
IS  - 1045-2257 (Print)
IS  - 1045-2257 (Linking)
VI  - 46
IP  - 10
DP  - 2007 Oct
TI  - Wilms' tumor with an apparently balanced translocation t(X;18) resulting in 
      deletion of the WTX gene.
PG  - 909-13
AB  - The recent description of a new X chromosome tumor suppressor gene, WTX, that is 
      commonly inactivated in Wilms' tumor prompted us to examine the possible 
      involvement of WTX in a case of Wilms' tumor containing an apparently balanced 
      reciprocal translocation between chromosomes X and 18 (t(X;18)(q11;p11)). 
      Fluorescence in situ hybridization (FISH) analysis of paraffin tumor sections 
      indeed revealed a deletion of the WTX locus at Xq11. High-resolution array 
      comparative genomic hybridization (array CGH) analysis of tumor DNA revealed a 
      1.5 Mb chromosome deletion encompassing the WTX gene at Xq11. No loss of genetic 
      material was detected on chromosome 18. Interestingly, unlike most tumors with 
      acquired chromosomal translocations, where a new fusion oncogene or 
      promoter-oncogene fusion is created and drives tumor growth, the t(X;18) in this 
      tumor appears to drive tumorigenesis via deletion of a tumor suppressor. This 
      case demonstrates the importance of array CGH and FISH as adjuncts in tumor 
      cytogenetics and in identifying pathogenic microdeletions in "balanced" 
      translocations that are not truly balanced.
CI  - Copyright (c) 2007 Wiley-Liss, Inc.
FAU - Han, Moonjoo
AU  - Han M
AD  - Molecular Diagnostics Laboratory, Department of Pathology, Massachusetts General 
      Hospital, and Harvard Medical School, Boston, MA 02114, USA.
FAU - Rivera, Miguel N
AU  - Rivera MN
FAU - Batten, Julie M
AU  - Batten JM
FAU - Haber, Daniel A
AU  - Haber DA
FAU - Dal Cin, Paola
AU  - Dal Cin P
FAU - Iafrate, A John
AU  - Iafrate AJ
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Genes Chromosomes Cancer
JT  - Genes, chromosomes & cancer
JID - 9007329
RN  - 0 (AMER1 protein, human)
RN  - 0 (Adaptor Proteins, Signal Transducing)
RN  - 0 (Tumor Suppressor Proteins)
SB  - IM
MH  - Adaptor Proteins, Signal Transducing
MH  - Chromosomes, Human, Pair 18/*genetics
MH  - Chromosomes, Human, X/*genetics
MH  - *Gene Deletion
MH  - Gene Expression Profiling
MH  - Humans
MH  - In Situ Hybridization, Fluorescence
MH  - Infant, Newborn
MH  - Karyotyping
MH  - Kidney Neoplasms/*genetics/metabolism
MH  - Male
MH  - Nucleic Acid Hybridization
MH  - Oligonucleotide Array Sequence Analysis
MH  - *Translocation, Genetic
MH  - Tumor Suppressor Proteins/*genetics
MH  - Wilms Tumor/*genetics/metabolism
EDAT- 2007/07/11 09:00
MHDA- 2007/10/27 09:00
CRDT- 2007/07/11 09:00
PHST- 2007/07/11 09:00 [pubmed]
PHST- 2007/10/27 09:00 [medline]
PHST- 2007/07/11 09:00 [entrez]
AID - 10.1002/gcc.20476 [doi]
PST - ppublish
SO  - Genes Chromosomes Cancer. 2007 Oct;46(10):909-13. doi: 10.1002/gcc.20476.