title: RNA-binding proteins in neurological diseases.
abstract: Emerging studies support that RNA-binding proteins (RBPs) play critical roles in human biology and pathogenesis. RBPs are essential players in RNA processing and metabolism, including pre-mRNA splicing, polyadenylation, transport, surveillance, mRNA localization, mRNA stability control, translational control and editing of various types of RNAs. Aberrant expression of and mutations in RBP genes affect various steps of RNA processing, altering target gene function. RBPs have been associated with various diseases, including neurological diseases. Here, we mainly focus on selected RNA-binding proteins including Nova-1/Nova-2, HuR/HuB/HuC/HuD, TDP-43, Fus, Rbfox1/Rbfox2, QKI and FMRP, discussing their function and roles in human diseases.
authors.forename: HuaLin
authors.initials: H
authors.lastname: Zhou
authors.forename: Marie
authors.initials: M
authors.lastname: Mangelsdorf
authors.forename: JiangHong
authors.initials: J
authors.lastname: Liu
authors.forename: Li
authors.initials: L
authors.lastname: Zhu
authors.forename: Jane Y
authors.initials: JY
authors.lastname: Wu
mesh_terms: D000818:Animals; D000951:Antigens, Neoplasm / Q000378:metabolism; D004268:DNA-Binding Proteins / Q000378:metabolism; D051959:ELAV Proteins / Q000378:metabolism; D051860:Fragile X Messenger Ribonucleoprotein 1 / Q000378:metabolism; D020869:Gene Expression Profiling; D005786:Gene Expression Regulation; D006801:Humans; D051379:Mice; D009154:Mutation; D009419:Nerve Tissue Proteins / Q000378:metabolism; D009420:Nervous System; D009422:Nervous System Diseases / Q000378:metabolism / Q000503:physiopathology*; D000090682:Neuro-Oncological Ventral Antigen; D012323:RNA Processing, Post-Transcriptional; D000072260:RNA Splicing Factors; D012333:RNA, Messenger / Q000378:metabolism; D034702:RNA-Binding Protein FUS / Q000378:metabolism; D016601:RNA-Binding Proteins / Q000378:metabolism / Q000502:physiology*
chemical_list: D000951:Antigens, Neoplasm; D004268:DNA-Binding Proteins; D051959:ELAV Proteins; D051860:Fragile X Messenger Ribonucleoprotein 1; D009419:Nerve Tissue Proteins; D000090682:Neuro-Oncological Ventral Antigen; D000072260:RNA Splicing Factors; D012333:RNA, Messenger; D034702:RNA-Binding Protein FUS; D016601:RNA-Binding Proteins; C490832:QKI protein, human; C455479:RBFOX1 protein, human