Human Molecular Genetics, Vol 7, 637-642, Copyright © 1998 by Oxford University Press
D Smedley, R Hamoudi, J Clark, W Warren, M Abdul-Rauf, G Somers, D Venter, K Fagan, C Cooper and J Shipley
A recently described atypical myeloproliferative disorder is invariably
associated with reciprocal translocations involving 8p11-12. The most
common rearrangement is a t(8;13)(p11;q11-12). Here we determine that this
translocation results in the fusion of the fibroblast growth factor
receptor 1 gene (FGFR1), a member of the receptor tyrosine kinase family at
8p11, to a novel gene at 13q11-12 designated RAMP . The predicted RAMP
protein exhibits strong homology to the product of a recently cloned
candidate gene for X-linked mental retardation, DXS6673E . We also provide
the first report of a novel, putative metal- binding motif, present as five
tandem repeats in both RAMP and DXS6673E. RT-PCR detected only one of the
two possible fusion transcripts, encoding a product in which the N-terminal
641 amino acids of RAMP become joined to the tyrosine kinase domain of
FGFR1. Receptor tyrosine kinases are not commonly involved in the formation
of tumour- specific fusion proteins. However, the previous reports of
involvement of receptor tyrosine kinases in fusion proteins in
non-Hodgkin's lymphoma, chronic myelomonocytic leukaemia and papillary
thyroid carcinoma described similar rearrangements. By analogy with these,
we propose that the RAMP-FGFR1 fusion product will contribute to
progression of this myeloproliferative disorder by constitutive activation
of tyrosine kinase function.
ARTICLES
The t(8;13)(p11;q11-12) rearrangement associated with an atypical myeloproliferative disorder fuses the fibroblast growth factor receptor 1 gene to a novel gene RAMP
Cell Biology and Experimental Pathology Section, Institute of Cancer Research, Haddow Laboratories, Belmont, Surrey SM2 5NG, UK. damian@icr.ac.uk
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