InterPro domain: IPR019148

This entry represents a family of proteins known variously as DiGeorge syndrome critical region 14 (DGCR14), DiGeorge syndrome protein I (DGSI), ES2 and ESS-2. In yeast the homologue is known as stress response protein Bis1. Proteins in this family consist of approximately 500 residues with alternating regions of low complexity and conservation where the domain similarities are strong. They contain two predicted coiled-coil regions. ESS-2 has been shown to be involved in pre-mRNA splicing [ 1 ]. Similarly DGCR14 has been shown to be a noncore component of the spliceosome C complex [ 2 ].

1. Systematic analyses of rpm-1 suppressors reveal roles for ESS-2 in mRNA splicing in Caenorhabditis elegans. Genetics 198, 1101-15
2. Dynamic protein-protein interaction wiring of the human spliceosome. Mol. Cell 45, 567-80