Hicks G G, Singh N, Nashabi A, Mai S, Bozek G, Klewes L, Arapovic D, White E K, Koury M J, Oltz E M, Van Kaer L, Ruley H E
Manitoba Institute of Cell Biology and the University of Manitoba, Winnipeg, Canada.
Nat Genet. 2000 Feb;24(2):175-9. doi: 10.1038/72842.
The gene FUS (also known as TLS (for translocated in liposarcoma) and hnRNP P2) is translocated with the gene encoding the transcription factor ERG-1 in human myeloid leukaemias. Although the functions of wild-type FUS are unknown, the protein contains an RNA-recognition motif and is a component of nuclear riboprotein complexes. FUS resembles a transcription factor in that it binds DNA, contributes a transcriptional activation domain to the FUS-ERG oncoprotein and interacts with several transcription factors in vitro. To better understand FUS function in vivo, we examined the consequences of disrupting Fus in mice. Our results indicate that Fus is essential for viability of neonatal animals, influences lymphocyte development in a non-cell-intrinsic manner, has an intrinsic role in the proliferative responses of B cells to specific mitogenic stimuli and is required for the maintenance of genomic stability. The involvement of a nuclear riboprotein in these processes in vivo indicates that Fus is important in genome maintenance.
基因FUS（也称为TLS（脂肉瘤中的易位基因）和hnRNP P2）在人类髓系白血病中与编码转录因子ERG-1的基因发生易位。尽管野生型FUS的功能尚不清楚，但该蛋白含有一个RNA识别基序，是核糖核蛋白复合物的一个组成部分。FUS类似于转录因子，因为它能结合DNA，为FUS-ERG癌蛋白提供转录激活结构域，并在体外与多种转录因子相互作用。为了更好地了解FUS在体内的功能，我们研究了破坏小鼠Fus基因的后果。我们的结果表明，Fus对新生动物的生存能力至关重要，以非细胞自主方式影响淋巴细胞发育，在B细胞对特定促有丝分裂刺激的增殖反应中具有内在作用，并且是维持基因组稳定性所必需的。一种核糖核蛋白在体内这些过程中的参与表明Fus在基因组维持中很重要。
