The Gemin6-Gemin7 heterodimer from the survival of motor neurons complex has an Sm protein-like structure

Yingli Ma; Josée Dostie; Gideon Dreyfuss; Gregory D Van Duyne

doi:10.1016/j.str.2005.03.014

The Gemin6-Gemin7 heterodimer from the survival of motor neurons complex has an Sm protein-like structure

Structure. 2005 Jun;13(6):883-92. doi: 10.1016/j.str.2005.03.014.

Authors

Yingli Ma¹, Josée Dostie, Gideon Dreyfuss, Gregory D Van Duyne

Affiliation

¹ Department of Biochemistry and Biophysics, Howard Hughes Medical Institute, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA.

PMID: 15939020
DOI: 10.1016/j.str.2005.03.014

Abstract

The survival of motor neurons (SMN) protein, product of the disease gene of the common neurodegenerative disease spinal muscular atrophy, is part of the large multiprotein "SMN complex." The SMN complex functions as an assembly machine for small nuclear ribonucleoproteins (snRNPs)-the major components of the spliceosome. Here, we report the crystal structure of two components of the human SMN complex, Gemin6 and Gemin7. Although Gemin6 and Gemin7 have no significant sequence similarity with Sm proteins, both adopt canonical Sm folds. Moreover, Gemin6 and Gemin7 exist as a heterodimer, and interact with each other via an interface similar to that which mediates interactions among the Sm proteins. Together with binding experiments that show that the Gemin6/Gemin7 complex binds to Sm proteins, these findings provide a framework for considering how the SMN complex, with Gemin6 and Gemin7 as tools, might organize Sm proteins for formation of Sm rings on snRNA targets.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Amino Acid Motifs
Amino Acid Sequence
Carrier Proteins / chemistry*
Carrier Proteins / drug effects
Carrier Proteins / isolation & purification
Carrier Proteins / metabolism
Conserved Sequence
Crystallography, X-Ray
Dimerization
Humans
Hydrogen Bonding
Hydrophobic and Hydrophilic Interactions
Models, Chemical
Models, Molecular
Molecular Sequence Data
Motor Neurons / chemistry*
Multiprotein Complexes / chemistry
Nuclear Proteins / chemistry*
Nuclear Proteins / drug effects
Nuclear Proteins / isolation & purification
Nuclear Proteins / metabolism
Protein Binding
Protein Folding
Protein Structure, Secondary
Protein Structure, Tertiary
Ribonucleoproteins, Small Nuclear / metabolism
SMN Complex Proteins
Sequence Homology, Amino Acid
Spliceosomes / chemistry
Static Electricity
Trypsin / pharmacology
X-Ray Diffraction

Substances

Carrier Proteins
GEMIN6 protein, human
Multiprotein Complexes
Nuclear Proteins
Ribonucleoproteins, Small Nuclear
SMN Complex Proteins
gemin7 protein, human
Trypsin

Associated data

PDB/1Y96