Letter by Seavey and Rubin Regarding Article, “Sustained Activation of Endothelial YAP1 Causes Epithelioid Hemangioendothelioma”

Seavey, Caleb N.; Rubin, Brian P.

doi:10.1161/atvbaha.121.316754

Cited by 1 publication

(1 citation statement)

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“…Overall, these two studies have elucidated the significant role played by the TFE3 portion of the fusion protein, as it alters the DNA-binding profile, transcriptional landscape, and phenotype of the resultant tumors. Therefore, considering this fusion to be an overactive form of YAP is an oversimplification [ 80 ].…”

Section: Understanding the Yap1–tfe3 Fusionmentioning

confidence: 99%

Unraveling the Biology of Epithelioid Hemangioendothelioma, a TAZ–CAMTA1 Fusion Driven Sarcoma

Seavey

Pobbati

Rubin

2022

Cancers

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The activities of YAP and TAZ, the end effectors of the Hippo pathway, are consistently altered in cancer, and this dysregulation drives aggressive tumor phenotypes. While the actions of these two proteins aid in tumorigenesis in the majority of cancers, the dysregulation of these proteins is rarely sufficient for initial tumor development. Herein, we present a unique TAZ-driven cancer, epithelioid hemangioendothelioma (EHE), which harbors a WWTR1(TAZ)–CAMTA1 gene fusion in at least 90% of cases. Recent investigations have elucidated the mechanisms by which YAP/TAP-fusion oncoproteins function and drive tumorigenesis. This review presents a critical evaluation of this recent work, with a particular focus on how the oncoproteins alter the normal activity of TAZ and YAP, and, concurrently, we generate a framework for how we can target the gene fusions in patients. Since EHE represents a paradigm of YAP/TAZ dysregulation in cancer, targeted therapies for EHE may also be effective against other YAP/TAZ-dependent cancers.

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Section: Understanding the Yap1–tfe3 Fusionmentioning

confidence: 99%