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. Author manuscript; available in PMC: 2016 Jun 1.
Published in final edited form as: Curr Opin Virol. 2015 Feb 9;12:20–25. doi: 10.1016/j.coviro.2015.01.011

Fig 4.

Fig 4

Model of how the gain-of-function (GOF) mutations in MDA5 cause Aicardi-Goutieres Syndrome (AGS). These mis-sense mutations over-stabilize the MDA5 filament, resulting in increased sensitivity to short dsRNAs and aberrant activation by as-yet-unidentified cellular RNAs.