Snord 3A: A Molecular Marker and Modulator of Prion Disease Progression

Eran Cohen, Dana Avrahami, Kati Frid, Tamar Canello, Ephrat Levy Lahad, Sharon Zeligson, Shira Perlberg, Joab Chapman, Oren S. Cohen, Esther Kahana, Iris Lavon, Ruth Gabizon*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

17 Scopus citations


Since preventive treatments for prion disease require early identification of subjects at risk, we searched for surrogate peripheral markers characterizing the asymptomatic phases of such conditions. To this effect, we subjected blood mRNA from E200K PrP CJD patients and corresponding family members to global arrays and found that the expression of Snord3A, a non-coding RNA transcript, was elevated several times in CJD patients as compared to controls, while asymptomatic carriers presented intermediate Snord3A levels. In the brains of TgMHu2ME199K mice, a mouse model mimicking for E200K CJD, Snord 3A levels were elevated in an age and disease severity dependent manner, as was the case for brains of these mice in which disease was exacerbated by copper administration. Snord3A expression was also elevated in scrapie infected mice, but not in PrP0/0 mice, indicating that while the expression levels of this transcript may reflect diverse prion etiologies, they are not related to the loss of PrPC's function. Elevation of Snord3A was consistent with the activation of ATF6, representing one of the arms of the unfolded protein response system. Indeed, SnoRNAs were associated with reduced resistance to oxidative stress, and with ER stress in general, factors playing a significant role in this and other neurodegenerative conditions. We hypothesize that in addition to its function as a disease marker, Snord3A may play an important role in the mechanism of prion disease manifestation and progression.

Original languageAmerican English
Article numbere54433
JournalPLoS ONE
Issue number1
StatePublished - 21 Jan 2013
Externally publishedYes


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