Chapter 5: Evolution of PRNP and SPRN
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Evolution of vertebrate genes related to prion and Shadoo proteins--clues from comparative genomic analysis.
Recent findings of new genes in fish related to the prion protein (PrP) gene PRNP, including our recent report of SPRN coding for Shadoo (Sho) protein found also in mammals, raise issues of their function and evolution. Here we report additional novel fish genes found in public databases, including a duplicated SPRN gene, SPRNB, in Fugu, Tetraodon, carp, and zebrafish encoding the Sho2 protein,...
متن کاملDifferential expression of Prnp and Sprn in scrapie infected sheep also reveals Prnp genotype specific differences.
The central role for PrP in the pathogenesis of the transmissible spongiform encephalopathies (TSEs) is illustrated by the resistance of Prnp(0/0) mice to disease and by the inverse association of Prnp gene dosage with incubation period. Understanding the role of PrP(C) in TSEs necessitates knowledge of expression levels of the Prnp gene during the development of disease. SSBP/1 scrapie shows a...
متن کاملFrequent Missense and Insertion/Deletion Polymorphisms in the Ovine Shadoo Gene Parallel Species-Specific Variation in PrP
BACKGROUND The cellular prion protein PrP(C) is encoded by the Prnp gene. This protein is expressed in the central nervous system (CNS) and serves as a precursor to the misfolded PrP(Sc) isoform in prion diseases. The prototype prion disease is scrapie in sheep, and whereas Prnp exhibits common missense polymorphisms for V136A, R154H and Q171R in ovine populations, genetic variation in mouse Pr...
متن کاملShadoo/PrP (Sprn0/0/Prnp0/0) double knockout mice
Shadoo (Sho) is a brain glycoprotein with similarities to the unstructured region of PrP (C) . Frameshift alleles of the Sho gene, Sprn, are reported in variant Creutzfeldt-Jakob disease (vCJD) patients while Sprn mRNA knockdown in PrP-null (Prnp(0/0) ) embryos produces lethality, advancing Sho as the hypothetical PrP-like "pi" protein. Also, Sho levels are reduced as misfolded PrP accumulates ...
متن کاملRole of the prion protein family in the gonads
The prion-gene family comprises four members named PRNP (PRP(c)), PRND (Doppel), PRNT (PRT), and SPRN (Shadoo). According to species, PRND is located 16-52 kb downstream from the PRNP locus, whereas SPRN is located on another chromosome. The fourth prion-family gene, PRNT, belongs to the same genomic cluster as PRNP and PRND in humans and bovidae. PRNT and PRND possibly resulted from a duplicat...
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