Unrip is a component of SMN complexes active in snRNP assembly

FEBS Lett. 2005 Apr 25;579(11):2348-54. doi: 10.1016/j.febslet.2005.03.034.

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

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Antibodies, Monoclonal / immunology
  • Antibodies, Monoclonal / pharmacology
  • Carrier Proteins / metabolism
  • Cyclic AMP Response Element-Binding Protein / antagonists & inhibitors
  • Cyclic AMP Response Element-Binding Protein / chemistry
  • Cyclic AMP Response Element-Binding Protein / immunology
  • Cyclic AMP Response Element-Binding Protein / metabolism*
  • Female
  • HeLa Cells
  • Humans
  • Mice
  • Mice, Inbred BALB C
  • Multiprotein Complexes / chemistry
  • Multiprotein Complexes / metabolism
  • Neoplasm Proteins / metabolism*
  • Nerve Tissue Proteins / antagonists & inhibitors
  • Nerve Tissue Proteins / chemistry
  • Nerve Tissue Proteins / immunology
  • Nerve Tissue Proteins / metabolism*
  • Nuclear Proteins / metabolism
  • Protein Binding
  • Protein Transport
  • RNA-Binding Proteins / antagonists & inhibitors
  • RNA-Binding Proteins / chemistry
  • RNA-Binding Proteins / immunology
  • RNA-Binding Proteins / metabolism*
  • Ribonucleoproteins, Small Nuclear / metabolism*
  • SMN Complex Proteins

Substances

  • Antibodies, Monoclonal
  • Carrier Proteins
  • Cyclic AMP Response Element-Binding Protein
  • GEMIN6 protein, human
  • Multiprotein Complexes
  • Neoplasm Proteins
  • Nerve Tissue Proteins
  • Nuclear Proteins
  • RNA-Binding Proteins
  • Ribonucleoproteins, Small Nuclear
  • SMN Complex Proteins
  • STRAP protein, human
  • gemin7 protein, human