Abstract
Metastasis is the most deadly aspect of cancer and results from acquired gene regulation abnormalities in tumor cells. Transcriptional regulation is an essential component of controlling of gene function and its failure could contribute to tumor progression and metastasis. During cancer progression, deregulation of oncogenic or tumor suppressive transcription factors, as well as master cell fate regulators, collectively influences multiple steps of the metastasis cascade, including local invasion and dissemination of the tumor to distant organs. Transcription factor PAX3/Pax3, which contributes to diverse cell lineages during embryonic development, plays a major role in tumorigenesis. Mutations in this gene can cause neurodevelopmental disease and the existing literature supports that there is a potential link between aberrant expression of PAX3 genes in adult tissues and a wide variety of cancers. PAX3 function is tissue-specific and could contribute to tumorigenesis either directly as oncogene or as a tumor suppressor by losing its function. In this review, we discuss comprehensively the differential role played by PAX3 in various tissues and how its aberrant expression is implicated in disease development. This review particularly highlights the oncogenic and tumor suppressor role played by PAX3 in different cancers and underlines the importance of precisely identifying tissue-specific role of PAX3 in order to determine its exact role in development of cancer.
Highlights
Cancer is a disease that is characterized by uncontrolled cell growth, proliferation, migration, and invasion of abnormal cells resulting in aggregation of these cells to form tumors in organ
PAX3 is a pivotal gene involved in organ development and is known to play a very important role in development of cancer
The studies involving ectopic expression of PAX3 in thyroid cancers show that it can act as a tumor suppressor
Summary
Cancer is a disease that is characterized by uncontrolled cell growth, proliferation, migration, and invasion of abnormal cells resulting in aggregation of these cells to form tumors in organ. The paired box (PAX: human)/(Pax: mouse) gene family is known to be associated with developmental functions and play a crucial role in cellular proliferation, differentiation, migration, and tissue development [2]. Loss of function of PAX3 due to decreased expression caused by hypermethylation is reasoned out as an underlying cause for development of thyroid cancer [36] Based on these facts, it is important to determine the functions of PAX3 specific to tissue type and understand its role in disease pathogenesis. This review will mainly discuss about the specific roles played by PAX3 in different tissue types and will focus on specific examples where PAX3 has contradictory function as either oncogene or tumor suppressor, and is implicated in development of cancer
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