| Primary Identifier | IPR027652 | Type | Family |
| Short Name | PRP8 |
| description | Pre-mRNA-processing-splicing factor 8 (Prp8) is a central component of the spliceosome, which may play a role in aligning the pre-mRNA 5'- and 3'-exons for ligation. It interacts with U5 snRNA, and with pre-mRNA 5'-splice sites in B spliceosomes and 3'-splice sites in C spliceosomes. It is part of the U5 snRNP complex, and of U5.4/6 and U5.U4atac/U6atac snRNP complexes in U2- and U12-dependent spliceosomes, respectively. It is also found in a mRNA splicing-dependent exon junction complex (EJC) with SRRM1 where it interacts with U5 snRNP proteins SNRP116 and WDR57/SPF38 [, ].Mutations of human Prp8 cause retinitis pigmentosa 13, a retinal dystrophy belonging to the group of pigmentary retinopathies []. |
