Homozygous inactivation of the NF1 gene in bone marrow cells from children with neurofibromatosis type 1 and malignant myeloid disorders.

@article{Side1997HomozygousIO,
  title={Homozygous inactivation of the NF1 gene in bone marrow cells from children with neurofibromatosis type 1 and malignant myeloid disorders.},
  author={Lucy E Side and Benjamin Taylor and Michelle Cayouette and Edward Conner and Peter Thompson and Marilynn Luce and Kevin M. Shannon},
  journal={The New England journal of medicine},
  year={1997},
  volume={336 24},
  pages={1713-20}
}
BACKGROUND The risk of malignant myeloid disorders in young children with neurofibromatosis type 1 is 200 to 500 times the normal risk. The gene for neurofibromatosis type 1 (NF1) encodes neurofibromin, a protein that negatively regulates signals transduced by Ras proteins. Genetic and biochemical data support the hypothesis that NF1 functions as a tumor-suppressor gene in immature myeloid cells, but inactivation of both NF1 alleles has not been demonstrated in leukemic cells from patients with… CONTINUE READING

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