Article
Crystal structure of the C-terminal domain of splicing factor Prp8 carrying retinitis pigmentosa mutants.
Protein science : a publication of the Protein Society - 1 Jun 2007
Zhang Lingdi, Shen Jingping, Guarnieri Michael T, Heroux Annie, Yang Kui, Zhao Rui
Abstract excerpt
Prp8 is a critical pre-mRNA splicing factor. Prp8 is proposed to help form and stabilize the spliceosome catalytic core and to be an important regulator of spliceosome activation. Mutations in human Prp8 (hPrp8) cause a severe form of the genetic disorder retinitis pigmentosa, RP13. Understanding the molecular mechanism of Prp8's function in pre-mRNA splicing and RP13 has been hindered by its large size (over...
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