The survival motor neuron protein of Schizosacharomyces pombe. Conservation of survival motor neuron interaction domains in divergent organisms.
Spinal muscular atrophy is a common often lethal neurodegenerative disease resulting from deletions or mutations in the survival motor neuron gene (SMN). SMN is ubiquitously expressed in metazoan cells and plays a role in small nuclear ribonucleoprotein assembly and pre-mRNA splicing. Here we characterize the Schizosacharomyces pombe orthologue of SMN (yeast SMN (ySMN)). We report that the ySMN protein is essential for viability and localizes in both the cytoplasm and the nucleus. Like human SMN, we show that ySMN can oligomerize. Remarkably, ySMN interacts directly with human SMN and Sm proteins. The highly conserved carboxyl-terminal domain of ySMN is necessary for the evolutionarily conserved interactions of SMN and required for cell viability. We also demonstrate that the conserved amino-terminal region of ySMN is not required for SMN and Sm binding but is critical for the housekeeping function of SMN.
Paushkin
S
S
Howard Hughes Medical Institute, Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104-6148, USA.
Charroux
B
B
Abel
L
L
Perkinson
R A
RA
Pellizzoni
L
L
Dreyfuss
G
G
D000595:Amino Acid Sequence; D002451:Cell Compartmentation; D002467:Cell Nucleus / Q000737:chemistry; D017124:Conserved Sequence; D017362:Cyclic AMP Response Element-Binding Protein; D003593:Cytoplasm / Q000737:chemistry; D019143:Evolution, Molecular; D005656:Fungal Proteins / Q000235:genetics* / Q000378:metabolism; D020043:Genes, Essential; D006801:Humans; D008969:Molecular Sequence Data; D009419:Nerve Tissue Proteins / Q000235:genetics* / Q000378:metabolism; D011485:Protein Binding; D011487:Protein Conformation; D016271:Proto-Oncogene Proteins c-myc / Q000378:metabolism; D016601:RNA-Binding Proteins; D055532:SMN Complex Proteins; D012568:Schizosaccharomyces / Q000235:genetics*; D013045:Species Specificity
D017362:Cyclic AMP Response Element-Binding Protein; D005656:Fungal Proteins; D009419:Nerve Tissue Proteins; D016271:Proto-Oncogene Proteins c-myc; D016601:RNA-Binding Proteins; D055532:SMN Complex Proteins