DEAD-box helicase 11 (DDX11)

DEAD-box helicase 11 (DDX11), also frequently referred to as ChlR1, is an ATP-dependent DNA helicase belonging to the DEAH/DEAD-box family that is essential for maintaining genomic stability (UniProt Q9H862). It plays a pivotal role in the establishment of sister chromatid cohesion during the S phase of the cell cycle and is involved in various DNA repair pathways, including the Fanconi anemia-like pathway and homologous recombination (PubMed: 20473336). DDX11 is specifically required for the resolution of complex DNA structures, such as G-quadruplexes, to ensure smooth replication fork progression and prevent DNA damage (PubMed: 27013501). Mutations in the DDX11 gene are the underlying cause of Warsaw breakage syndrome, a rare genetic disorder characterized by microcephaly, pre- and postnatal growth retardation, and abnormal skin pigmentation (OMIM: 601150). In the context of oncology, DDX11 is often overexpressed in multiple cancer types, including melanoma, lung cancer, and osteosarcoma, where it contributes to tumor cell proliferation, survival, and resistance to DNA-damaging agents (PubMed: 32670183). While there are currently no approved drugs targeting DDX11, it is considered an emerging therapeutic target in cancer research, particularly for strategies involving synthetic lethality in tumors with existing DNA repair deficiencies.

Other names

CHL1ChlR1Keratinocyte-associated protein 1KCAP1Probable ATP-dependent RNA helicase DDX11

Inhibition of ATP-dependent DNA unwinding and G-quadruplex resolution