|
|
 Previous Article | Table of Contents | Next Article 
Blood, Vol. 92 No. 5 (September 1), 1998:
pp. 1735-1742
Consistent Fusion of ZNF198 to the Fibroblast Growth Factor
Receptor-1 in the t(8;13)(p11;q12) Myeloproliferative Syndrome
Andreas Reiter,
Jastinder Sohal,
Shashikant Kulkarni,
Andrew Chase,
Donald H.C. Macdonald,
Ricardo C.T. Aguiar,
Cristina Gonçalves,
Jesus M. Hernandez,
Barbara A. Jennings,
John M. Goldman, and
Nicholas C.P. Cross
From the Department of Haematology, Imperial College School of
Medicine, Hammersmith Hospital, London, UK; the Dana Farber Cancer
Institute, Boston, MA; the Hospital Geral de Santo António,
Oporto, Portugal; the Hospital Universitario de Salamanca, Salamanca,
Spain; and the Norfolk and Norwich Hospital, Norwich, UK.
The 8p11 myeloproliferative syndrome is a rare, aggressive condition
associated with reciprocal translocations of chromosome band 8p11, most
commonly the t(8;13)(p11;q12). To identify the genes involved in this
translocation, we used fluorescence in situ hybridization (FISH)
analysis to show that the chromosome 8 breakpoints fell within YAC
899e2 and that the chromosome 13 breakpoints are clustered in a region
flanked by YACs 929f11 and 911h8. FISH using chromosome 13 PAC clones
indicated that the t(8;13) is not simply a reciprocal translocation but
also involves an inversion of 13q11-12. Exon trapping of a PAC that
spanned the chromosome 13 translocation breakpoints led to the
identification of a gene, ZNF198, that detected rearranged bands when
used as a probe against Southern blots of patient DNA. Conceptual
translation of the full-length ZNF198 cDNA sequence predicts a protein
of 1377 amino acids that shows significant homology to the
DXS6673E/KIAA0385 and KIAA0425 proteins. Alignment of these three
proteins revealed a novel, conserved Zn-finger-related motif (MYM
domain) of the general form
CX2C19-22CX3CX13-19CX2CX19-25FCX3CX3F/Y
that is repeated five times in each protein. To identify the
translocation partner gene on chromosome 8, 5 and
3 RACE polymerase chain reactions (PCRs) were
performed on patient RNA with several combinations of ZNF198 primers.
Clones were identified in which the ZNF198 was fused to exon 9 of the
fibroblast growth factor receptor-1 (FGFR1), a gene known to map to
8p11. An identical ZNF198-FGFR1 fusion was detected in the three
patients with a t(8;13) for whom RNA was available; reciprocal
FGFR1-ZNF198 transcripts were not detected. Rearrangements of both
ZNF198 and FGFR1 were found in two further patients by Southern
blotting. ZNF198-FGFR1 includes the five MYM domains of ZNF198 and the
intracellular tyrosine kinase domain of FGFR1. We hypothesize that this
fusion leads to constitutive activation of the FGFR1 tyrosine kinase in
a manner analogous to the activation of ABL by BCR in chronic myeloid
leukemia.
© 1998 by The American Society of Hematology.
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
A. Dutt, H. B. Salvesen, T.-H. Chen, A. H. Ramos, R. C. Onofrio, C. Hatton, R. Nicoletti, W. Winckler, R. Grewal, M. Hanna, et al.
Drug-sensitive FGFR2 mutations in endometrial carcinoma
PNAS,
June 24, 2008;
105(25):
8713 - 8717.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
C. Walz, G. Metzgeroth, C. Haferlach, A. Schmitt-Graeff, A. Fabarius, V. Hagen, O. Prummer, S. Rauh, R. Hehlmann, A. Hochhaus, et al.
Characterization of three new imatinib-responsive fusion genes in chronic myeloproliferative disorders generated by disruption of the platelet-derived growth factor receptor {beta} gene
Haematologica,
February 1, 2007;
92(2):
163 - 169.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. Ren, D. Raina, W. Chen, G. Li, L. Huang, and D. Kufe
MUC1 Oncoprotein Functions in Activation of Fibroblast Growth Factor Receptor Signaling
Mol. Cancer Res.,
November 1, 2006;
4(11):
873 - 883.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
P. W. Finch and J. S. Rubin
Keratinocyte growth factor expression and activity in cancer: implications for use in patients with solid tumors.
J Natl Cancer Inst,
June 21, 2006;
98(12):
812 - 824.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Reiter, C. Walz, A. Watmore, C. Schoch, I. Blau, B. Schlegelberger, U. Berger, N. Telford, S. Aruliah, J. A. Yin, et al.
The t(8;9)(p22;p24) Is a Recurrent Abnormality in Chronic and Acute Leukemia that Fuses PCM1 to JAK2
Cancer Res.,
April 1, 2005;
65(7):
2662 - 2667.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
T. Maeda, F. Yagasaki, M. Ishikawa, N. Takahashi, and M. Bessho
Transforming property of TEL-FGFR3 mediated through PI3-K in a T-cell lymphoma that subsequently progressed to AML
Blood,
March 1, 2005;
105(5):
2115 - 2123.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
C. Heath and N. C. P. Cross
Critical Role of STAT5 Activation in Transformation Mediated by ZNF198-FGFR1
J. Biol. Chem.,
February 20, 2004;
279(8):
6666 - 6673.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. M. Goldman and J. V. Melo
Chronic Myeloid Leukemia -- Advances in Biology and New Approaches to Treatment
N. Engl. J. Med.,
October 9, 2003;
349(15):
1451 - 1464.
[Full Text]
[PDF]
|
|
|
|
|
|
|
G. Guasch, C. Popovici, F. Mugneret, M. Chaffanet, P. Pontarotti, D. Birnbaum, and M.-J. Pebusque
Endogenous retroviral sequence is fused to FGFR1 kinase in the 8p12 stem-cell myeloproliferative disorder with t(8;19)(p12;q13.3)
Blood,
January 1, 2003;
101(1):
286 - 288.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
K. Shchors, F. Yehiely, R. K. Kular, K. U. Kotlo, G. Brewer, and L. P. Deiss
Cell Death Inhibiting RNA (CDIR) Derived from a 3'-Untranslated Region Binds AUF1 and Heat Shock Protein 27
J. Biol. Chem.,
November 27, 2002;
277(49):
47061 - 47072.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. Kong, C. S. Wang, and D. J. Donoghue
Interaction of Fibroblast Growth Factor Receptor 3 and the Adapter Protein SH2-B. A ROLE IN STAT5 ACTIVATION
J. Biol. Chem.,
May 3, 2002;
277(18):
15962 - 15970.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Demiroglu, E. J. Steer, C. Heath, K. Taylor, M. Bentley, S. L. Allen, P. Koduru, J. P. Brody, G. Hawson, R. Rodwell, et al.
The t(8;22) in chronic myeloid leukemia fuses BCR to FGFR1: transforming activity and specific inhibition of FGFR1 fusion proteins
Blood,
December 15, 2001;
98(13):
3778 - 3783.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
G. Guasch, V. Ollendorff, J.-P. Borg, D. Birnbaum, and M.-J. Pebusque
8p12 Stem Cell Myeloproliferative Disorder: the FOP-Fibroblast Growth Factor Receptor 1 Fusion Protein of the t(6;8) Translocation Induces Cell Survival Mediated by Mitogen-Activated Protein Kinase and Phosphatidylinositol 3-Kinase/Akt/mTOR Pathways
Mol. Cell. Biol.,
December 1, 2001;
21(23):
8129 - 8142.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. Schwaller, E. Anastasiadou, D. Cain, J. Kutok, S. Wojiski, I. R. Williams, R. LaStarza, B. Crescenzi, D. W. Sternberg, P. Andreasson, et al.
H4(D10S170), a gene frequently rearranged in papillary thyroid carcinoma, is fused to the platelet-derived growth factor receptor {beta} gene in atypical chronic myeloid leukemia with t(5;10)(q33;q22)
Blood,
June 15, 2001;
97(12):
3910 - 3918.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
K. C. Hart, S. C. Robertson, and D. J. Donoghue
Identification of Tyrosine Residues in Constitutively Activated Fibroblast Growth Factor Receptor 3 Involved in Mitogenesis, Stat Activation, and Phosphatidylinositol 3-Kinase Activation
Mol. Biol. Cell,
April 1, 2001;
12(4):
931 - 942.
[Abstract]
[Full Text]
|
|
|
|
|
|
|
N. A. Heerema, H. N. Sather, M. G. Sensel, M. K. Lee, R. J. Hutchinson, J. B. Nachman, G. H. Reaman, B. J. Lange, P. G. Steinherz, B. C. Bostrom, et al.
Abnormalities of Chromosome Bands 13q12 to 13q14 in Childhood Acute Lymphoblastic Leukemia
J. Clin. Oncol.,
November 15, 2000;
18(22):
3837 - 3844.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. W. N. Deininger, J. M. Goldman, and J. V. Melo
The molecular biology of chronic myeloid leukemia
Blood,
November 15, 2000;
96(10):
3343 - 3356.
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. Xiao, J. G. McCarthy, J. C. Aster, and J. A. Fletcher
ZNF198-FGFR1 transforming activity depends on a novel proline-rich ZNF198 oligomerization domain
Blood,
July 15, 2000;
96(2):
699 - 704.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. Kulkarni, C. Heath, S. Parker, A. Chase, S. Iqbal, C. F. Pocock, J. Kaeda, K. Cwynarski, J. M. Goldman, and N. C. P. Cross
Fusion of H4/D10S170 to the Platelet-derived Growth Factor Receptor {beta} in BCR-ABL-negative Myeloproliferative Disorders with a t(5;10)(q33;q21)
Cancer Res.,
July 1, 2000;
60(13):
3592 - 3598.
[Abstract]
[Full Text]
|
|
|
|
|
|
|
G. Guasch, G. J. Mack, C. Popovici, N. Dastugue, D. Birnbaum, J. B. Rattner, and M.-J. Pebusque
FGFR1 is fused to the centrosome-associated protein CEP110 in the 8p12 stem cell myeloproliferative disorder with t(8;9)(p12;q33)
Blood,
March 1, 2000;
95(5):
1788 - 1796.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
V. Ollendorff, G. Guasch, D. Isnardon, R. Galindo, D. Birnbaum, and M.-J. Pebusque
Characterization of FIM-FGFR1, the Fusion Product of the Myeloproliferative Disorder-associated t(8;13) Translocation
J. Biol. Chem.,
September 17, 1999;
274(38):
26922 - 26930.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
C. Popovici, B. Zhang, M.-J. Gregoire, P. Jonveaux, M. Lafage-Pochitaloff, D. Birnbaum, and M.-J. Pebusque
The t(6;8)(q27;p11) Translocation in a Stem Cell Myeloproliferative Disorder Fuses a Novel Gene, FOP, to Fibroblast Growth Factor Receptor 1
Blood,
February 15, 1999;
93(4):
1381 - 1389.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
