Strand exchange of telomeric DNA catalyzed by the Werner syndrome protein (WRN) is specifically stimulated by TRF2.

[werner syndrome]

Werner syndrome (WS ), caused by loss of function of the RecQ helicase WRN , is a hereditary disease characterized by premature aging and elevated cancer incidence . WRN has DNA binding , exonuclease , ATPase , helicase and strand annealing activities , suggesting possible roles in recombination-related processes . Evidence indicates that WRN deficiency causes telomeric abnormalities that likely underlie early onset of aging phenotypes in WS . Furthermore , TRF 2 , a protein essential for telomere protection , interacts with WRN and influences its basic helicase and exonuclease activities . However , these studies provided little insight into WRN 's specific function at telomeres . Here , we explored the possibility that WRN and TRF 2 cooperate during telomeric recombination processes . Our results indicate that TRF 2 , through its interactions with both WRN and telomeric DNA , stimulates WRN -mediated strand exchange specifically between telomeric substrates ; TRF 2 's basic domain is particularly important for this stimulation . Although TRF 1 binds telomeric DNA with similar affinity , it has minimal effects on WRN -mediated strand exchange of telomeric DNA . Moreover , TRF 2 is displaced from telomeric DNA by WRN , independent of its ATPase and helicase activities . Together , these results suggest that TRF 2 and WRN act coordinately during telomeric recombination processes , consistent with certain telomeric abnormalities associated with alteration of WRN function .

Diseases
Validation

Diseases presenting "certain telomeric abnormalities" symptom

werner syndrome

You can validate or delete this automatically detected symptom