title: [Structural study of polyglutamine tract-binding protein 1].
abstract: Polyglutamine tract-binding protein 1 (PQBP1) is a nuclear protein that regulates transcription and pre-mRNA splicing. In addition, the mutations in the PQBP1 gene are known to cause hereditary mental retardation. This review summarizes current knowledge about the solution structure of PQBP1. PQBP1 is an intrinsically disordered protein: its polar-rich domain and C-terminal domain are disordered under physiological conditions. PQBP1 binds to its target molecule U5-15kD via a continuous 23-residue segment of the C-terminal domain. The function of PQBP1 in the pre-mRNA splicing is also discussed.
authors: Faculty of Pharmaceutical Sciences, University of Toyama. mineyuki@pha.u-toyama.ac.jp
authors: Mineyuki
authors: M
authors: Mizuguchi
authors: Hitoshi
authors: H
authors: Okazawa
mesh_terms: D000595:Amino Acid Sequence; D000818:Animals; D006801:Humans; D008607:Intellectual Disability / Q000235:genetics*; D064267:Intrinsically Disordered Proteins; D008969:Molecular Sequence Data; D009154:Mutation; D009842:Oligopeptides / Q000737:chemistry* / Q000235:genetics* / Q000378:metabolism / Q000502:physiology; D011485:Protein Binding; D011487:Protein Conformation; D017434:Protein Structure, Tertiary; D012322:RNA Precursors / Q000235:genetics; D012326:RNA Splicing / Q000235:genetics; D017415:Ribonucleoprotein, U5 Small Nuclear / Q000378:metabolism; D014158:Transcription, Genetic
chemical_list: D064267:Intrinsically Disordered Proteins; D009842:Oligopeptides; D012322:RNA Precursors; D017415:Ribonucleoprotein, U5 Small Nuclear; C506012:TXNL4A protein, human; C407346:polyglutamine-binding protein 1