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- Tiffany R. Merlinsky
- 1Human Oncology and Pathogenesis Program, Memorial Sloan Kettering Cancer Center, New York, New York.
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- Ross L. Levine
- 1Human Oncology and Pathogenesis Program, Memorial Sloan Kettering Cancer Center, New York, New York.
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- Elodie Pronier
- 1Human Oncology and Pathogenesis Program, Memorial Sloan Kettering Cancer Center, New York, New York.
抄録
<jats:title>Abstract</jats:title> <jats:p>In 2013, two seminal studies identified gain-of-function mutations in the Calreticulin (CALR) gene in a subset of JAK2/MPL-negative myeloproliferative neoplasm (MPN) patients. CALR is an endoplasmic reticulum (ER) chaperone protein that normally binds misfolded proteins in the ER and prevents their export to the Golgi and had never previously been reported mutated in cancer or to be associated with hematologic disorders. Further investigation determined that mutated CALR is able to achieve oncogenic transformation primarily through constitutive activation of the MPL–JAK–STAT signaling axis. Here we review our current understanding of the role of CALR mutations in MPN pathogenesis and how these insights can lead to innovative therapeutics approaches.</jats:p>
収録刊行物
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- Clinical Cancer Research
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Clinical Cancer Research 25 (10), 2956-2962, 2019-05-15
American Association for Cancer Research (AACR)