DOI: 10.1073/pnas.94.10.5302. Acute leukemia with promyelocytic features in PML/RARα transgenic mice

Acute leukemia with promyelocytic features in PML/RARα transgenic mice

10.1073/pnas.94.10.5302

Crossref journal-article

Proceedings of the National Academy of Sciences

Proceedings of the National Academy of Sciences (341)

Abstract

Acute promyelocytic leukemia (APL) is associated with reciprocal chromosomal translocations involving the retinoic acid receptor α (RARα) locus on chromosome 17. In the majority of cases, RARα translocates and fuses with the promyelocytic leukemia (PML) gene located on chromosome 15. The resulting fusion genes encode the two structurally unique PML/RARα and RARα/PML fusion proteins as well as aberrant PML gene products, the respective pathogenetic roles of which have not been elucidated. We have generated transgenic mice in which the PML/RARα fusion protein is specifically expressed in the myeloid-promyelocytic lineage. During their first year of life, all the PML/RARα transgenic mice have an abnormal hematopoiesis that can best be described as a myeloproliferative disorder. Between 12 and 14 months of age, 10% of them develop a form of acute leukemia with a differentiation block at the promyelocytic stage that closely mimics human APL even in its response to retinoic acid. Our results are conclusive in vivo evidence that PML/RARα plays a crucial role in the pathogenesis of APL.

Bibliography

He, L.-Z., Tribioli, C., Rivi, R., Peruzzi, D., Pelicci, P. G., Soares, V., Cattoretti, G., & Pandolfi, P. P. (1997). Acute leukemia with promyelocytic features in PML/RARÎ±âtransgenicâmice. Proceedings of the National Academy of Sciences, 94(10), 5302â5307.

Authors 8

Li-Zhen He (first)
Carla Tribioli (additional)
Roberta Rivi (additional)
Daniela Peruzzi (additional)
Pier Giuseppe Pelicci (additional)
Vera Soares (additional)
Giorgio Cattoretti (additional)
Pier Paolo Pandolfi (additional)

References 42 Referenced 301

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Dates

Type	When
Created	23 years, 1 month ago (July 26, 2002, 10:31 a.m.)
Deposited	3 years, 4 months ago (April 13, 2022, 2:43 p.m.)
Indexed	3 weeks, 2 days ago (Aug. 3, 2025, 6:59 p.m.)
Issued	28 years, 3 months ago (May 13, 1997)
Published	28 years, 3 months ago (May 13, 1997)
Published Online	28 years, 3 months ago (May 13, 1997)
Published Print	28 years, 3 months ago (May 13, 1997)

Funders 0

None

BibTeX

@article{He_1997, title={Acute leukemia with promyelocytic features in PML/RARα transgenic mice}, volume={94}, ISSN={1091-6490}, url={http://dx.doi.org/10.1073/pnas.94.10.5302}, DOI={10.1073/pnas.94.10.5302}, number={10}, journal={Proceedings of the National Academy of Sciences}, publisher={Proceedings of the National Academy of Sciences}, author={He, Li-Zhen and Tribioli, Carla and Rivi, Roberta and Peruzzi, Daniela and Pelicci, Pier Giuseppe and Soares, Vera and Cattoretti, Giorgio and Pandolfi, Pier Paolo}, year={1997}, month=may, pages={5302–5307} }

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      "name": "published",
      "label": "Published",
      "group": {
        "name": "publication_history",
        "label": "Publication History"
      }
    }
  ]
}