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.
Publication types
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Research Support, N.I.H., Extramural
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Research Support, Non-U.S. Gov't
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Review
MeSH terms
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Animals
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Antigens, Neoplasm / metabolism
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DNA-Binding Proteins / metabolism
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ELAV Proteins / metabolism
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Fragile X Mental Retardation Protein / metabolism
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Gene Expression Profiling
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Gene Expression Regulation*
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Humans
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Mice
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Mutation
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Nerve Tissue Proteins / metabolism
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Nervous System
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Nervous System Diseases / metabolism
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Nervous System Diseases / physiopathology*
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Neuro-Oncological Ventral Antigen
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RNA Processing, Post-Transcriptional
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RNA Splicing Factors
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RNA, Messenger / metabolism
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RNA-Binding Protein FUS / metabolism
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RNA-Binding Proteins / metabolism
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RNA-Binding Proteins / physiology*
Substances
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Antigens, Neoplasm
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DNA-Binding Proteins
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ELAV Proteins
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Nerve Tissue Proteins
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Neuro-Oncological Ventral Antigen
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QKI protein, human
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RBFOX1 protein, human
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RNA Splicing Factors
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RNA, Messenger
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RNA-Binding Protein FUS
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RNA-Binding Proteins
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Fragile X Mental Retardation Protein