title: The splicing factor U1-70K interacts with the SMN complex and is required for nuclear gem integrity.
abstract: The nuclear SMN complex localizes to specific structures called nuclear gems. The loss of gems is a cellular marker for several neurodegenerative diseases. Here, we identify that the U1-snRNP-specific protein U1-70K localizes to nuclear gems, and we show that U1-70K is necessary for gem integrity. Furthermore, we show that the interaction between U1-70K and the SMN complex is RNA independent, and we map the SMN complex binding site to the unstructured N-terminal tail of U1-70K. Consistent with these results, the expression of the U1-70K N-terminal tail rescues gem formation. These findings show that U1-70K is an SMN-complex-associating protein, and they suggest a new function for U1-70K in the formation of nuclear gems.
authors: Department of RNA Biology, Institute of Molecular Genetics AS CR, 142 20 Prague, Czech Republic Faculty of Science, Charles University in Prague, 128 43 Prague, Czech Republic.
authors: Eva
authors: E
authors: Stejskalová
authors: Department of RNA Biology, Institute of Molecular Genetics AS CR, 142 20 Prague, Czech Republic david.stanek@img.cas.cz.
authors: David
authors: D
authors: Staněk
mesh_terms: D001665:Binding Sites; D002467:Cell Nucleus / Q000737:chemistry / Q000235:genetics / Q000378:metabolism; D055531:Gemini of Coiled Bodies / Q000737:chemistry / Q000378:metabolism*; D006367:HeLa Cells; D006801:Humans; D011485:Protein Binding; D021381:Protein Transport; D012326:RNA Splicing; D017412:Ribonucleoprotein, U1 Small Nuclear / Q000737:chemistry / Q000235:genetics / Q000378:metabolism*; D055532:SMN Complex Proteins / Q000235:genetics / Q000378:metabolism*
chemical_list: D017412:Ribonucleoprotein, U1 Small Nuclear; D055532:SMN Complex Proteins; C490688:SNRNP70 protein, human
keywords: Cajal bodies; Gems; Nuclear structure; SMN; U1 snRNP; U1-70K