Affordable Access

Publisher Website

Shadoo/PrP (Sprn0/0/Prnp0/0) double knockout mice : More than zeroes

Authors
Journal
Prion
1933-6896
Publisher
Landes Bioscience
Publication Date
Volume
6
Issue
5
Identifiers
DOI: 10.4161/pri.21867
Keywords
  • Extra View
Disciplines
  • Biology
  • Medicine

Abstract

Shadoo (Sho) is a brain glycoprotein with similarities to the unstructured region of PrPC. Frameshift alleles of the Sho gene, Sprn, are reported in variant Creutzfeldt-Jakob disease (vCJD) patients while Sprn mRNA knockdown in PrP-null (Prnp0/0) embryos produces lethality, advancing Sho as the hypothetical PrP-like “pi” protein. Also, Sho levels are reduced as misfolded PrP accumulates during prion infections. To penetrate these issues we created Sprn null alleles (Daude et al., Proc. Natl. Acad. Sci USA 2012; 109(23): 9035–40). Results from the challenge of Sprn null and TgSprn transgenic mice with rodent-adapted prions coalesce to define downregulation of Sho as a “tracer” for the formation of misfolded PrP. However, classical BSE and rodent-adapted BSE isolates may behave differently, as they do for other facets of the pathogenic process, and this intriguing variation warrants closer scrutiny. With regards to physiological function, double knockout mice (Sprn0/0/Prnp0/0) mice survived to over 600 d of age. This suggests that Sho is not pi, or, given the accumulating data for many activities for PrPC, that the pi hypothesis invoking a discrete signaling pathway to maintain neuronal viability is no longer tenable.

There are no comments yet on this publication. Be the first to share your thoughts.

Statistics

Seen <100 times
0 Comments

More articles like this

Metallothionein-I/II double knockout mice are no m...

on International journal of toxic... 2005

Is there more to TSE diseases than PrP?

on Trends in Microbiology April 2000

PRP: more words than facts...

on Knee Surgery Sports Traumatolo... September 2012
More articles like this..