本日は、mRNAの品質管理異常に起因する免疫応答の活性化が行動的・電気生理学的異常を引き起こす論文について勉強しました。

Inhibition of Upf2-Dependent Nonsense-Mediated Decay Leads to Behavioral and Neurophysiological Abnormalities by Activating the Immune Response

Jennifer L.Johnson¹²²⁷LoredanaStoica²³²⁷YuweiLiu¹²²⁷Ping JunZhu¹²AbhisekBhattacharya⁴⁵Shelly A.Buffington¹²RedwanHuq⁶N. TonyEissa⁴⁵OlaLarsson⁷Bo T.Porse⁸⁹¹⁰DeeptiDomingo¹¹UrwahNawaz¹²ReneeCarroll¹²LachlanJolly¹²Tom S.Scerri¹³Hyung-GooKim¹⁴AmandaBrignell¹⁵Matthew J.Coleman¹⁶…MauroCosta-Mattioli¹²³²⁸

Neuron. 2019 Oct 1. pii: S0896-6273(19)30733-0. doi: 10.1016/j.neuron.2019.08.027. [Epub ahead of print]

Highlights
• Humans carrying novel variants in UPF2 exhibit speech and language deficits

• Upf2-deficient mice and flies have impaired NMD and exhibit behavioral deficits

• Inhibition of Upf2-dependent NMD triggers immune activation in mice

• Reduction of brain inflammation reverses synaptic and behavioral deficits

論文紹介：ST