HepA-相关蛋白——一种复制叉结合因子

HepA-related protein: a replication fork binding factor

HepA-相关蛋白(HepA-related protein,HARP),又名SMARCAL1(SWI/SNF-related,matrix-associated,actin-depen-dent regulator of chromatin,subfamily a-like 1),是一种ATP驱动的退火解旋酶,催化解旋的单链DNA重新缠绕成双螺旋。HARP突变可造成一种多系统常染色体隐性疾病——Schimke免疫-骨发育不良(Schimke immuno-osseous dysplasia,SIOD)的发生。HARP可与复制蛋白A(replication protein A,PRA)直接相互作用而被招募到DNA损伤位点以稳定停滞的复制叉,从而保持基因组的完整性。这些研究展示了HARP是DNA损伤反应中的一个关键元件,并且对SIOD发病机制的理解具有重要意义。

HARP （HepA-related protein）,also known as SMARCAL1 （SWI/SNF-related,matrix-associated,actin-dependent regulator of chromatin,subfamily a-like 1）,is an ATP-driven annealing helicase which catalyzes the rewinding of the stably unwound DNA.Mutations in HARP are a cause of Schimke immuno-osseous dysplasia （SIOD）,a multisystem autosomal recessive disorder.HARP can interact directly with RPA （replication protein A） and is recruited to the sites of DNA damage to stabilize stalled replication forks,so as to maintain genome integrity.These researches reveal HARP is a key component in DNA damage response and are beneficial to understanding of SIOD.