Familial neurofibromatosis and juvenile chronic myelogenous leukemia

Clark, Robin D.; Hutter, John J.

doi:10.1007/bf00303009

Cited by 31 publications

(3 citation statements)

References 15 publications

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“…NF1 patients have a significantly increased risk of developing malignancies such as neurofibrosarcomas (e.g., see reference 51). They also have been reported to have a higher incidence of juvenile myeloid leukemia (2,12). The postulated role of Evi-2 in murine neoplastic disease, combined with its chromosomal location in humans and the increased frequency of malignancies observed in patients with NF1, makes Evi-2 a good candidate for the NF1 gene.…”

Section: Discussionmentioning

confidence: 97%

Evi-2, a common integration site involved in murine myeloid leukemogenesis.

Buchberg¹,

Bedigian²,

Jenkins³

et al. 1990

Mol. Cell. Biol.

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BXH-2 mice have the highest incidence of spontaneous retrovirally induced myeloid leukemia of any known inbred strain and, as such, represent a valuable model system for identifying cellular proto-oncogenes involved in myeloid disease. Chronic murine leukemia viruses often induce disease by insertional activation or mutation of cellular proto-oncogenes. These loci are identified as common viral integration sites in tumor DNAs. Here we report on the characterization of a novel common viral integration site in BXH-2 myeloid leukemias, designated Evi-2. Within the cluster of viral integration sites that define Evi-2, we identified a gene that has the potential for encoding a novel protein of 223 amino acids. This putative proto-oncogene possesses all of the structural features of a transmembrane protein. Within the transmembrane domain is a "leucine zipper," suggesting that Evi-2 is involved in either homopolymer or heteropolymer formation, which may play an important role in the normal functioning of Evi-2. Interestingly, the human homolog of Evi-2 has recently been shown to be tightly linked to the von Recklinghausen neurofibromatosis locus, suggesting a role for Evi-2 in human disease as well.

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Section: Discussionmentioning

confidence: 97%

Evi-2, a common integration site involved in murine myeloid leukemogenesis.

Buchberg¹,

Bedigian²,

Jenkins³

et al. 1990

Mol. Cell. Biol.

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“…Up to 75 % of cases of JMML have a disorder in the GM-CSF/Ras signal transduction pathway (Emanuel 2004). In fact, the most common myeloid malignancy in children with NF type 1 is JMML, which seems to develop in a stepwise fashion (Clark and Hutter 1982;Miles et al 1996;Leung et al 2003).…”

Section: Pathogenesismentioning

confidence: 99%

Myeloid Neoplasms with a Monocytoid Lineage

Zimmermann¹

2016

Tumors and Tumor-Like Lesions of the Hepatobiliary Tract

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“…8 Clark and Hutter reported an apparent association between the rare entity juvenile chronic myelogenous leukemia and neurofibromatosis. 9 They suggested that other types of nonlymphocytic leukemia have an increased frequency, but Riccardi raised the question as to whether these are families with only café au lait spots. 10 Voutsinas and Wynne-Davies suggested that the risk of malignant change in neurofibromatosis had been exaggerated 11 and reported the accurate figure to be 2.0% (or 4.2% of those older than 21 years of age).…”

Section: Signs and Symptomsmentioning

confidence: 99%