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中枢神经系统糖蛋白Shadoo具有类似朊蛋白（PrP(C)）的保护特性，并且在朊病毒感染中水平降低。

The CNS glycoprotein Shadoo has PrP(C)-like protective properties and displays reduced levels in prion infections.

作者信息

Watts Joel C, Drisaldi Bettina, Ng Vivian, Yang Jing, Strome Bob, Horne Patrick, Sy Man-Sun, Yoong Larry, Young Rebecca, Mastrangelo Peter, Bergeron Catherine, Fraser Paul E, Carlson George A, Mount Howard T J, Schmitt-Ulms Gerold, Westaway David

机构信息

Centre for Research in Neurodegenerative Diseases, University of Toronto, Toronto, Canada.

出版信息

EMBO J. 2007 Sep 5;26(17):4038-50. doi: 10.1038/sj.emboj.7601830. Epub 2007 Aug 16.

DOI:10.1038/sj.emboj.7601830
PMID:17703189
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1950727/
Abstract

The cellular prion protein, PrP(C), is neuroprotective in a number of settings and in particular prevents cerebellar degeneration mediated by CNS-expressed Doppel or internally deleted PrP ('DeltaPrP'). This paradigm has facilitated mapping of activity determinants in PrP(C) and implicated a cryptic PrP(C)-like protein, 'pi'. Shadoo (Sho) is a hypothetical GPI-anchored protein encoded by the Sprn gene, exhibiting homology and domain organization similar to the N-terminus of PrP. Here we demonstrate Sprn expression and Sho protein in the adult CNS. Sho expression overlaps PrP(C), but is low in cerebellar granular neurons (CGNs) containing PrP(C) and high in PrP(C)-deficient dendritic processes. In Prnp(0/0) CGNs, Sho transgenes were PrP(C)-like in their ability to counteract neurotoxic effects of either Doppel or DeltaPrP. Additionally, prion-infected mice exhibit a dramatic reduction in endogenous Sho protein. Sho is a candidate for pi, and since it engenders a PrP(C)-like neuroprotective activity, compromised neuroprotective activity resulting from reduced levels may exacerbate damage in prion infections. Sho may prove useful in deciphering several unresolved facets of prion biology.

摘要

细胞朊蛋白PrP(C)在多种情况下具有神经保护作用，尤其能预防由中枢神经系统表达的多配体蛋白聚糖（Doppel）或内部缺失的PrP（“DeltaPrP”）介导的小脑变性。这一模式有助于确定PrP(C)中的活性决定因素，并涉及一种隐秘的类PrP(C)蛋白“π”。影子蛋白（Shadoo，Sho）是一种由Sprn基因编码的假定糖基磷脂酰肌醇（GPI）锚定蛋白，与PrP的N端具有相似的同源性和结构域组织。在此，我们展示了Sprn在成体中枢神经系统中的表达及Sho蛋白。Sho的表达与PrP(C)重叠，但在含有PrP(C)的小脑颗粒神经元（CGN）中水平较低，而在缺乏PrP(C)的树突状突起中水平较高。在Prnp(0/0) CGN中，Sho转基因在对抗Doppel或DeltaPrP的神经毒性作用方面具有类似PrP(C)的能力。此外，朊病毒感染的小鼠内源性Sho蛋白显著减少。Sho是“π”的候选蛋白，由于它具有类似PrP(C)的神经保护活性，水平降低导致的神经保护活性受损可能会加重朊病毒感染中的损伤。Sho可能有助于破解朊病毒生物学中几个尚未解决的方面。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/66fa/1994130/19c7488bd8b4/7601830f7.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/66fa/1994130/6b8e52d997fa/7601830f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/66fa/1994130/1a9d3e5a7bec/7601830f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/66fa/1994130/a81eeb6c79a1/7601830f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/66fa/1994130/0832caddba2c/7601830f4ah.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/66fa/1994130/ba5756c93383/7601830f4ip.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/66fa/1994130/fdfe17900a4f/7601830f5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/66fa/1994130/e33493601b55/7601830f6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/66fa/1994130/19c7488bd8b4/7601830f7.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/66fa/1994130/6b8e52d997fa/7601830f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/66fa/1994130/1a9d3e5a7bec/7601830f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/66fa/1994130/a81eeb6c79a1/7601830f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/66fa/1994130/0832caddba2c/7601830f4ah.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/66fa/1994130/ba5756c93383/7601830f4ip.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/66fa/1994130/fdfe17900a4f/7601830f5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/66fa/1994130/e33493601b55/7601830f6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/66fa/1994130/19c7488bd8b4/7601830f7.jpg

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