[HTML][HTML] Rrs1 is involved in endoplasmic reticulum stress response in Huntington disease
A Carnemolla, E Fossale, E Agostoni… - Journal of Biological …, 2009 - ASBMB
The induction of Rrs1 expression is one of the earliest events detected in a presymptomatic
knock-in mouse model of Huntington disease (HD). Rrs1 up-regulation fulfills the HD criteria
of dominance, striatal specificity, and polyglutamine dependence. Here we show that
mammalian Rrs1 is localized both in the nucleolus as well as in the endoplasmic reticulum
(ER) of neurons. This dual localization is shared with its newly identified molecular partner
3D3/lyric. We then show that both genes are induced by ER stress in neurons. Interestingly …
knock-in mouse model of Huntington disease (HD). Rrs1 up-regulation fulfills the HD criteria
of dominance, striatal specificity, and polyglutamine dependence. Here we show that
mammalian Rrs1 is localized both in the nucleolus as well as in the endoplasmic reticulum
(ER) of neurons. This dual localization is shared with its newly identified molecular partner
3D3/lyric. We then show that both genes are induced by ER stress in neurons. Interestingly …