期刊
FEBS LETTERS
卷 579, 期 11, 页码 2348-2354出版社
WILEY
DOI: 10.1016/j.febslet.2005.03.034
关键词
survival of motor neurons; spinal muscular atrophy; small nuclear ribonucleoprotein; unr-interacting protein
资金
- Telethon [TCP02011] Funding Source: Medline
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. (c) 2005 Federation of European Biochemical Societies. Published by Elsevier B.V. All rights reserved.
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