Fig. 7
From: Staufen1 links RNA stress granules and autophagy in a model of neurodegeneration
Silencing of STAU1 mitigates SCA2 phenotypes. a Stau1 haploinsufficiency improves abnormal motor behavior of ATXN2Q127 mice as determined by rotarod behavior at 8, 12, 16, and 20 weeks of age. ATXN2Q127;Stau1+/- mice (green) have improved rotarod performance compared with ATXN2Q127 littermates (red) starting at 12 weeks of age. Note that Stau1 haploinsufficiency (orange) by itself does not alter motor function; n = 9–15 mice per group. Values shown are mean ± SE. Significance was determined using generalized estimating equations (GEE). NS, nonsignificant, *P < 0.05, **P < 0.01. b, c Reduction of Stau1 in vivo improves levels of key cerebellar proteins towards normalization. b Western blotting of cerebellar extracts from ATXN2Q127;Stau1+/- mice showing improvement of protein levels for Calb1, Pcp2, Rgs8, Pcp4, Homer3, and Fam107b towards normalization. Each lane represents cerebellar extract from an individual mouse. β-Actin is used as a loading control and the blots are from three replicate experiments. c Quantitative analysis of western blots shown in b. Data are mean ± SD, **P < 0.01, ***P < 0.001, Student t-test. d Combined immunostaining of ATXN2 (red) and Stau1 (green) of cerebellar sections from ATXN2Q127 and crossed ATXN2Q127;Stau1+/- mice (34 weeks of age) demonstrating reduced ATXN2-Stau1 aggregates in crossed ATXN2Q127;Stau1+/- mice. Scale bar, 30 µM. e Model for STAU1 in the pathology of SCA2 and other neurodegenerative diseases
