In this, an increase of the p.7M allele was found in sCJD, and codon 7 is predicted to be contained within the signal peptide sequence of SPRN. The frequency of these alleles in healthy controls is strongly suggestive that no deleterious transport of Sho occurs due to methionine at residue 7 of the signal sequence, but this does not obviate the effect of subtle influences on protein transport upon establishment and progression of prion disease. The gene discussed is SPRN; the disease is prion disease.