Shadoo, a new protein highly conserved from fish to mammals and with similarity to prion protein

Genetic analysis of the SPRN gene in ruminants reveals polymorphisms in the alanine-rich segment of shadoo protein

“…1). There was no variation in the number of the basic Ala-ArgGly-type repeats within the ruminant Sho, but they differed from the rodent sequence (Premzl et al, 2003).…”

“…The mammalian prion protein gene family currently contains three known genes: PRNP (encoding prion protein, PrP), SPRN (encoding shadoo protein, Sho) and PRND (encoding doppel protein, Dpl) (Premzl et al, 2003(Premzl et al, , 2004Premzl & Gamulin, 2007;Watts & Westaway, 2007). PrP and Sho are highly expressed in the neurons of the central nervous system (Hu et al, 2007;, whereas Dpl appears to be specific for the reproductive system (Genoud et al, 2003).…”

New insights into cellular prion protein (PrPc) functions: The “ying and yang” of a relevant protein

“…A new gene named SPRN (shadow of prion protein) was described in 2003 (Premzl et al, 2003). SPRN comprises two exons, with the open reading frame (ORF) contained within exon 2, and codes for a protein of 130-150 amino acids named Shadoo (Sho, Japanese shadow) present in mammals as well as other vertebrates (Premzl et al, 2004).…”

Unchanged survival rates of Shadoo knockout mice after infection with mouse-adapted scrapie

“…Sprn, the gene encoding Sho, is conserved in mammals. 8 Based on its similarities to PrP C , which is implicated in prion disease pathogenesis, we hypothesized that Sho also plays an important role in prion diseases. This was suggested by the fact that 2 patients with early-onset variant Creutzfeldt-Jakob disease were found to harbor inactive Sprn alleles.…”

“…6,8 Previous work has shown overlapping expression patterns of Sho and PrP C in the central nervous system; like PrP C , Sho transgenes can counter the neurotoxic effects of Dpl, N-terminal truncated PrP C and reduced levels of Sho in experimental models of prion infection.…”