Oshima Junko, Huang Shurong, Pae Chong, Campisi Judith, Schiestl Robert H
Department of Pathology, University of Washington, Seattle, Washington, 98195, USA.
Cancer Res. 2002 Jan 15;62(2):547-51.
Loss of WRN causes the genomic instability progeroid syndrome, Werner syndrome. WRN encodes a multifunctional nuclear protein with 3'-->5' exonuclease and 3'-->5' helicase activities. Linear plasmids with noncompatible ends introduced to Werner syndrome cells underwent extensive deletions at nonhomologous joining ends, particularly at the 3' protruding single-stranded end. This extensive deletion phenotype was complemented by wild-type WRN. These results suggest that WRN can out-compete other exonucleases that participate in double-strand break repair or stabilize the broken DNA end.
WRN基因的缺失会导致基因组不稳定早衰综合征，即沃纳综合征。WRN编码一种具有3'→5'核酸外切酶和3'→5'解旋酶活性的多功能核蛋白。导入沃纳综合征细胞的具有不兼容末端的线性质粒在非同源末端连接时发生广泛缺失，特别是在3'突出单链末端。这种广泛的缺失表型可被野生型WRN互补。这些结果表明，WRN可以在竞争中胜出，超过其他参与双链断裂修复或稳定断裂DNA末端的核酸外切酶。
