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.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Animals
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Antibodies, Monoclonal / immunology
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Antibodies, Monoclonal / pharmacology
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Carrier Proteins / metabolism
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Cyclic AMP Response Element-Binding Protein / antagonists & inhibitors
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Cyclic AMP Response Element-Binding Protein / chemistry
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Cyclic AMP Response Element-Binding Protein / immunology
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Cyclic AMP Response Element-Binding Protein / metabolism*
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Female
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HeLa Cells
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Humans
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Mice
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Mice, Inbred BALB C
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Multiprotein Complexes / chemistry
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Multiprotein Complexes / metabolism
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Neoplasm Proteins / metabolism*
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Nerve Tissue Proteins / antagonists & inhibitors
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Nerve Tissue Proteins / chemistry
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Nerve Tissue Proteins / immunology
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Nerve Tissue Proteins / metabolism*
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Nuclear Proteins / metabolism
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Protein Binding
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Protein Transport
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RNA-Binding Proteins / antagonists & inhibitors
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RNA-Binding Proteins / chemistry
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RNA-Binding Proteins / immunology
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RNA-Binding Proteins / metabolism*
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Ribonucleoproteins, Small Nuclear / metabolism*
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SMN Complex Proteins
Substances
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Antibodies, Monoclonal
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Carrier Proteins
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Cyclic AMP Response Element-Binding Protein
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GEMIN6 protein, human
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Multiprotein Complexes
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Neoplasm Proteins
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Nerve Tissue Proteins
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Nuclear Proteins
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RNA-Binding Proteins
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Ribonucleoproteins, Small Nuclear
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SMN Complex Proteins
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STRAP protein, human
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gemin7 protein, human