Loss of the normal NF1 allele from the bone marrow of children with type 1 neurofibromatosis and malignant myeloid disorders.

@article{Shannon1994LossOT,
  title={Loss of the normal NF1 allele from the bone marrow of children with type 1 neurofibromatosis and malignant myeloid disorders.},
  author={Kevin M. Shannon and Peter O'Connell and George A. Martin and D C Paderanga and Karen Olson and Patricia Anne Dinndorf and Frank Mccormick},
  journal={The New England journal of medicine},
  year={1994},
  volume={330 9},
  pages={597-601}
}
BACKGROUND Children with type 1 neurofibromatosis (NF-1) are at increased risk for malignant myeloid disorders. Analysis of the NF-1 gene (NF1) suggests that the function of its product, neurofibromin, is reduced in affected persons and that NF1 belongs to the tumor-suppressor class of recessive cancer genes. This model is consistent with evidence that neurofibromin accelerates the intrinsic guanosine triphosphate-hydrolyzing activity of the Ras family of regulatory proteins. Loss of… CONTINUE READING

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