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Fusion Oncoproteins in Childhood Cancers: Potential Role in Targeted Therapy
Author(s) -
Sara D A Angione,
Alemayehu Akalu,
Jessica Gartrell,
Elimika Pfuma Fletcher,
Gilbert J. Burckart,
Gregory H. Reaman,
Ruby Leong,
Clinton F. Stewart
Publication year - 2021
Publication title -
the journal of pediatric pharmacology and therapeutics
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.456
H-Index - 15
eISSN - 2331-348X
pISSN - 1551-6776
DOI - 10.5863/1551-6776-26.6.541
Subject(s) - fusion gene , disease , targeted therapy , carcinogenesis , pediatric cancer , cancer , medicine , childhood cancer , cancer research , bioinformatics , biology , gene , genetics , pathology
Cancer remains the leading cause of death from disease in children. Historically, in contrast to their adult counterparts, the causes of pediatric malignancies have remained largely unknown, with most pediatric cancers displaying low mutational burdens. Research related to molecular genetics in pediatric cancers is advancing our understanding of potential drivers of tumorigenesis and opening new opportunities for targeted therapies. One such area is fusion oncoproteins, which are a product of chromosomal rearrangements resulting in the fusion of different genes. They have been identified as oncogenic drivers in several sarcomas and leukemias. Continued advancement in the understanding of the biology of fusion oncoproteins will contribute to the discovery and development of new therapies for childhood cancers. Here we review the current scientific knowledge on fusion oncoproteins, focusing on pediatric sarcomas and hematologic cancers, and highlight the challenges and current efforts in developing drugs to target fusion oncoproteins.

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