作者: Shu Yang , Sadaf T. Warraich , Garth A. Nicholson , Ian P. Blair
DOI: 10.1016/J.BIOCEL.2010.06.003
关键词: RNA 、 DNA 、 Transcriptional regulation 、 RNA recognition motif 、 Genetics 、 Gene 、 Zinc finger 、 Biology 、 Molecular biology 、 RNA-binding protein 、 Messenger RNA
摘要: Abstract The fused in sarcoma/translocated liposarcoma (FUS/TLS) gene was initially identified as a component of fusion pro-oncogene resulting from chromosomal translocation seen liposarcomas. FUS/TLS belongs to sub-family RNA binding proteins, encoding an N-terminal serine–tyrosine–glycine–glutamine (SYGQ) region, recognition motif (RRM) flanked by glycine rich (G-rich) regions, cysteine2/cysteine2 zinc finger and multiple RGG repeats. protein interacts with RNA, single stranded DNA double DNA, is involved unique functions mRNA processing transport, transcriptional regulation maintenance genomic stability. Recently, several mutations this have been found amyotrophic lateral sclerosis (ALS) patients. mutant forms exhibit similar pathology other ALS causative genes, including aberrant cytoplasmic inclusions increased nuclear ratio. patients suggests that altered metabolism may play role pathogenesis.