Prp8 Retinitis Pigmentosa Mutants Cause Defects in the Transition between the Catalytic Steps of Splicing

ABSTRACT: The essential process of pre-mRNA splicing must occur with high fidelity and efficiency for proper gene expression. The spliceosome employs DExD/H box helicases to promote on-pathway interactions while simultaneously minimizing errors. Prp8 and Snu114, an EF2-like GTPase, regulate the activity of the Brr2 helicase, promoting RNA unwinding by Brr2 at appro-priate points in the splicing cycle and repressing it at others. Mutations linked to Retinitis Pig-mentosa (RP), a disease that causes blindness in humans, map to the Brr2 regulatory region of Prp8. Previous In vitro studies of homologous mutations in Saccharomyces cerevisiae show that Prp8-RP mutants cause defects in spliceosome activation. Here we show a subset of RP muta-tions in Prp8 also cause defects in the transition between the 1s

ORGANISM(S): Saccharomyces cerevisiae

SUBMITTER: Megan Mayerle 

PROVIDER: E-GEOD-75081 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress