Abstract
DDX11/ChlR1 is a super-family two iron–sulfur cluster containing DNA helicase with roles in DNA replication and sister chromatid cohesion establishment, and general chromosome architecture. Bi-allelic mutations of the DDX11 gene cause a rare hereditary disease, named Warsaw breakage syndrome, characterized by a complex spectrum of clinical manifestations (pre- and post-natal growth defects, microcephaly, intellectual disability, heart anomalies and sister chromatid cohesion loss at cellular level) in accordance with the multifaceted, not yet fully understood, physiological functions of this DNA helicase. In the last few years, a possible role of DDX11 in the onset and progression of many cancers is emerging. Herein we summarize the results of recent studies, carried out either in tumoral cell lines or in xenograft cancer mouse models, suggesting that DDX11 may have an oncogenic role. The potential of DDX11 DNA helicase as a pharmacological target for novel anti-cancer therapeutic interventions, as inferred from these latest developments, is also discussed.
Highlights
DNA helicases are enzymes able to unwind DNA duplexes and translocate along nucleic acid strands with a specific directionality (30 to 50 or vice versa) by utilizing the energy derived from nucleoside triphosphate hydrolysis [1,2]
In a review article published in 2015 by Pearl and collaborators about the identification of DNA damage response proteins that are deregulated in various cancers and which could represent novel important therapeutic targets, DDX11 was mentioned as a potential oncogene [75]
A gene that has >20% truncating/inactivating mutations in cancer tissues can be considered a tumor suppressor genes (TSGs); if a gene has >20% missense mutations in recurrent positions, it can be considered as an OG [76]
Summary
DNA helicases are enzymes able to unwind DNA duplexes and translocate along nucleic acid strands with a specific directionality (30 to 50 or vice versa) by utilizing the energy derived from nucleoside triphosphate hydrolysis (typically ATP) [1,2]. DNA helicases play key functions in a variety of DNA replication/repair/recombination pathways that are important for genome stability maintenance. It is, not surprising that mutations and/or dysregulated expressions of DNA helicase encoding genes are linked to many hereditary diseases, neurodegenerative disorders, and cancers [3]. DNA helicases may help highly proliferating ual, indicating a pro-tumorigenic behavior of the corresponding enzymes In this regard, it cells to counteract theDNA replication stress from the conflicts between iscancer well established that certain helicases mayderiving help highly proliferating cancer cellsthe replication and transcription machineries, and to repair lesions due to either to counteract the replication stress deriving from the conflicts between the replication endogenous or exogenous insults and transcription machineries, and [4,5,6]. Dedicated to the role DDX11 is believed to play in various cancer types
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
