Myxoid liposarcoma: it's a hippo's world

Abstract Fused in sarcoma: DNA damage‐inducible transcript 3 protein (FUS:DDIT3) is a chimeric fusion oncoprotein present in 90% of human myxoid liposarcomas (MLS). The study by Trautmann et al in this issue of EMBO Molecular Medicine utilizes a drop‐out RNAi screen to establish hyperactive Yes‐asso...

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Main Authors: Carla Regina, Simone Hettmer
Format: Article
Language:English
Published: Springer Nature 2019-04-01
Series:EMBO Molecular Medicine
Online Access:https://doi.org/10.15252/emmm.201910470
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author Carla Regina
Simone Hettmer
author_facet Carla Regina
Simone Hettmer
author_sort Carla Regina
collection DOAJ
description Abstract Fused in sarcoma: DNA damage‐inducible transcript 3 protein (FUS:DDIT3) is a chimeric fusion oncoprotein present in 90% of human myxoid liposarcomas (MLS). The study by Trautmann et al in this issue of EMBO Molecular Medicine utilizes a drop‐out RNAi screen to establish hyperactive Yes‐associated protein 1 (YAP1), a major downstream nuclear effector of the Hippo signaling pathway, as a selectively essential transcript promoting viability and growth of MLS. These observations add to a growing body of evidence underscoring the importance of dysregulation of Hippo signaling in soft‐tissue sarcomas expressing fusion oncoproteins and identify a novel target for therapeutic intervention in MLS. Comprehensive molecular characterization pipelines are needed to screen patients with advanced soft‐tissue sarcomas for the presence of druggable alterations, including but not limited to nuclear YAP1 expression in MLS, to facilitate treatment decisions and advance therapy.
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spelling doaj-art-0d15cfd0238d4d53a606cf439bc868402025-08-20T03:06:00ZengSpringer NatureEMBO Molecular Medicine1757-46761757-46842019-04-011151310.15252/emmm.201910470Myxoid liposarcoma: it's a hippo's worldCarla Regina0Simone Hettmer1Division of Pediatric Hematology and Oncology, Department of Pediatric and Adolescent Medicine, University Medical Center Freiburg, University of FreiburgDivision of Pediatric Hematology and Oncology, Department of Pediatric and Adolescent Medicine, University Medical Center Freiburg, University of FreiburgAbstract Fused in sarcoma: DNA damage‐inducible transcript 3 protein (FUS:DDIT3) is a chimeric fusion oncoprotein present in 90% of human myxoid liposarcomas (MLS). The study by Trautmann et al in this issue of EMBO Molecular Medicine utilizes a drop‐out RNAi screen to establish hyperactive Yes‐associated protein 1 (YAP1), a major downstream nuclear effector of the Hippo signaling pathway, as a selectively essential transcript promoting viability and growth of MLS. These observations add to a growing body of evidence underscoring the importance of dysregulation of Hippo signaling in soft‐tissue sarcomas expressing fusion oncoproteins and identify a novel target for therapeutic intervention in MLS. Comprehensive molecular characterization pipelines are needed to screen patients with advanced soft‐tissue sarcomas for the presence of druggable alterations, including but not limited to nuclear YAP1 expression in MLS, to facilitate treatment decisions and advance therapy.https://doi.org/10.15252/emmm.201910470
spellingShingle Carla Regina
Simone Hettmer
Myxoid liposarcoma: it's a hippo's world
EMBO Molecular Medicine
title Myxoid liposarcoma: it's a hippo's world
title_full Myxoid liposarcoma: it's a hippo's world
title_fullStr Myxoid liposarcoma: it's a hippo's world
title_full_unstemmed Myxoid liposarcoma: it's a hippo's world
title_short Myxoid liposarcoma: it's a hippo's world
title_sort myxoid liposarcoma it s a hippo s world
url https://doi.org/10.15252/emmm.201910470
work_keys_str_mv AT carlaregina myxoidliposarcomaitsahipposworld
AT simonehettmer myxoidliposarcomaitsahipposworld