DNA replication fork stabilization factor DONSON (DONSON)

DNA replication fork stabilization factor DONSON (DONSON) is a highly conserved replisome-associated protein essential for stabilizing DNA replication forks during genome duplication and for maintaining genome stability[1][2][4][5]. DONSON participates in the initiation of DNA replication by promoting assembly of the active CMG helicase at replication origins via interaction with TopBP1 in a CDK-dependent manner[4][5]. Following initiation, DONSON remains associated with the replisome during elongation, where it interacts with key replication proteins (such as MCM helicase, GINS complex, Cdc45, PCNA, and Treslin) to maintain fork stability—even under replication stress[2][3][4][5]. Deficiency or genetic mutation of DONSON results in severely increased fork stalling, decreased checkpoint activity, replication-associated DNA double strand breaks, and chromosomal instability, causing clinical syndromes including microcephalic dwarfism and Meier-Gorlin syndrome[1][2][5]. Current research positions DONSON as a candidate disease gene relevant to cancer, developmental disorders, and genome maintenance, but no existing drugs directly target DONSON as of 2024[1][2][4][5].

Other names

Protein downstream neighbor of SonC21orf60B17MIMISMISSLADKFZP434M035downstream neighbor of SON