Prion diseases, or transmissible spongiform encephalopathies (TSEs), are manifested as genetic, infectious or sporadic lethal neurodegenerative disorders involving alterations of the prion protein (PrP). Constitutively expressed in normal adult brain, cellular PrP (PrP(C)) is sensitive to proteinase K digestion and is converted to the disease form, PrPSc, through alterations in protein folding conformation, which make it resistant proteases. SPRN (shadow of prion protein), also known as SHO or SHADOO, is a 151 amino acid cytoplasmic protein that is mainly expressed in brain. SPRN is considered a prion-like protein that has PrP(C)-like neuroprotective activity and may act as a modulator for the biological actions of normal and abnormal PrP. In humans, mutations in the gene encoding SPRN may be associated with variant and sporadic Creutzfeldt-Jakob disease, a degenerative neurological disorder that is incurable and invariably fatal
Function:
Prion-like protein that has PrP(C)-like neuroprotective activity. May act as a modulator for the biological actions of normal and abnormal PrP.
Subcellular Location:
Cell membrane; Lipid-anchor, GPI-anchor.
Tissue Specificity:
Mainly expressed in brain (at protein level). In brain, it is highly expressed in the hippocampus and cerebellum and is also expressed at lower level in other areas of the brain including the cerebral cortex, the thalamus and the medulla. In hippocampus and cerebellum it is highly expressed in the cell bodies of pyramidal cells and Purkinje cells, respectively.
Post-translational modifications:
N-glycosylated.
Similarity:
Belongs to the SPRN family.
SWISS:
Q8BWU1
Gene ID:
212518
Database links:
Entrez Gene: 503542 Human
Entrez Gene: 212518 Mouse
Omim: 610447 Human
SwissProt: Q5BIV9 Human
SwissProt: Q8BWU1 Mouse
Unigene: 64968 Human
Unigene: 246858 Mouse
朊病毒蛋白（朊蛋白相关蛋白、沙杜蛋白）是导致动物脑细胞死亡的传染性病毒蛋白，是一种致病性神经蛋白。
朊蛋白相关蛋白Shadoo是近期才被发行的新朊蛋白，其传播、传染过程尚不清楚，发病机理还有待于研究中。
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