title: Unrip is a component of SMN complexes active in snRNP assembly.
abstract: A macromolecular complex containing survival of motor neurons (SMN), the spinal muscular atrophy protein, and Gemin2-7 interacts with Sm proteins and snRNAs to carry out the assembly of these components into spliceosomal small nuclear ribonucleoproteins (snRNPs). Here we report the characterization of unr-interacting protein (unrip), a GH-WD protein of unknown function, as a component of the SMN complex that interacts directly with Gemin6 and Gemin7. Unrip also binds a subset of Sm proteins, and unrip-containing SMN complexes are necessary and sufficient to mediate the assembly of spliceosomal snRNPs. These results demonstrate that unrip functions in the pathway of snRNP biogenesis and is a marker of cellular SMN complexes active in snRNP assembly.
authors.affiliation: Dulbecco Telethon Institute, Institute of Cell Biology (CNR), Monterotondo Scalo, Rome, Italy.
authors.forename: Claudia
authors.initials: C
authors.lastname: Carissimi
authors.forename: Jennifer
authors.initials: J
authors.lastname: Baccon
authors.forename: Marco
authors.initials: M
authors.lastname: Straccia
authors.forename: Pieranna
authors.initials: P
authors.lastname: Chiarella
authors.forename: Alessio
authors.initials: A
authors.lastname: Maiolica
authors.forename: Alan
authors.initials: A
authors.lastname: Sawyer
authors.forename: Juri
authors.initials: J
authors.lastname: Rappsilber
authors.forename: Livio
authors.initials: L
authors.lastname: Pellizzoni
mesh_terms: D000818:Animals; D000911:Antibodies, Monoclonal / Q000276:immunology / Q000494:pharmacology; D002352:Carrier Proteins / Q000378:metabolism; D017362:Cyclic AMP Response Element-Binding Protein / Q000037:antagonists & inhibitors / Q000737:chemistry / Q000276:immunology / Q000378:metabolism*; D005260:Female; D006367:HeLa Cells; D006801:Humans; D051379:Mice; D008807:Mice, Inbred BALB C; D046912:Multiprotein Complexes / Q000737:chemistry / Q000378:metabolism; D009363:Neoplasm Proteins / Q000378:metabolism*; D009419:Nerve Tissue Proteins / Q000037:antagonists & inhibitors / Q000737:chemistry / Q000276:immunology / Q000378:metabolism*; D009687:Nuclear Proteins / Q000378:metabolism; D011485:Protein Binding; D021381:Protein Transport; D016601:RNA-Binding Proteins / Q000037:antagonists & inhibitors / Q000737:chemistry / Q000276:immunology / Q000378:metabolism*; D017411:Ribonucleoproteins, Small Nuclear / Q000378:metabolism*; D055532:SMN Complex Proteins
chemical_list: D000911:Antibodies, Monoclonal; D002352:Carrier Proteins; D017362:Cyclic AMP Response Element-Binding Protein; D046912:Multiprotein Complexes; D009363:Neoplasm Proteins; D009419:Nerve Tissue Proteins; D009687:Nuclear Proteins; D016601:RNA-Binding Proteins; D017411:Ribonucleoproteins, Small Nuclear; D055532:SMN Complex Proteins; C450836:GEMIN6 protein, human; C403447:STRAP protein, human; C466231:GEMIN7 protein, human