title: Brr2p RNA helicase with a split personality: insights into structure and function.
abstract: RNA helicases are involved in many cellular processes. Pre-mRNA splicing requires eight different DExD/H-box RNA helicases, which facilitate spliceosome assembly and remodelling of the intricate network of RNA rearrangements that are central to the splicing process. Brr2p, one of the spliceosomal RNA helicases, stands out through its unusual domain architecture. In the present review we highlight the advances made by recent structural and biochemical studies that have important implications for the mechanism and regulation of Brr2p activity. We also discuss the involvement of human Brr2 in retinitis pigmentosa, a degenerative eye disease, and how its functions in splicing might connect to the molecular pathology of the disease.
authors.affiliation: Wellcome Trust Centre for Cell Biology, University of Edinburgh, King's Buildings, Mayfield Road, Edinburgh EH9 3JR, Scotland, UK.
authors.forename: Daniela
authors.initials: D
authors.lastname: Hahn
authors.forename: Jean D
authors.initials: JD
authors.lastname: Beggs
mesh_terms: D000818:Animals; D002352:Carrier Proteins / Q000378:metabolism / Q000502:physiology; D005136:Eye Proteins / Q000737:chemistry / Q000378:metabolism / Q000502:physiology; D006801:Humans; D008869:Microtubule-Associated Proteins; D008954:Models, Biological; D008958:Models, Molecular; D020365:RNA Helicases / Q000737:chemistry / Q000378:metabolism / Q000502:physiology; D016601:RNA-Binding Proteins; D017415:Ribonucleoprotein, U5 Small Nuclear / Q000378:metabolism / Q000502:physiology; D017411:Ribonucleoproteins, Small Nuclear / Q000737:chemistry* / Q000378:metabolism / Q000502:physiology*; D013329:Structure-Activity Relationship
chemical_list: D002352:Carrier Proteins; D005136:Eye Proteins; D008869:Microtubule-Associated Proteins; C115374:PRPF8 protein, human; D016601:RNA-Binding Proteins; C120042:RP1 protein, human; D017415:Ribonucleoprotein, U5 Small Nuclear; D017411:Ribonucleoproteins, Small Nuclear; C545711:SNRNP200 protein, human; D020365:RNA Helicases