Fig. 2
From: Staufen1 links RNA stress granules and autophagy in a model of neurodegeneration
Staufen1 protein but not mRNA steady-state levels are increased in neurodegenerative disease cells and tissues. Western blot analysis of SCA2- FBs (a) and LBCs (b) show increased STAU1 levels compared with normal controls. DDX6 levels are unchanged. HD and SCA3 patient (polyQ expanded) FBs were used as additional controls. Four normal and five SCA2 FBs, and two normal and three SCA2 LBCs were used. c, d Western blot analyses of ATXN2Q127 (c) and BAC-Q72 (d) mouse cerebellar extracts (24 weeks of age) showing increased Stau1 levels compared with wild-type or BAC-Q22 controls (n = 2–3 animals per group). e Western blot of FB extracts from an ALS patient with the TDP-43G298S mutation show increased STAU1 levels. β-Actin was used as loading control and representative blots of three independent experiments are shown. f–h STAU1 RNA levels are unaltered in SCA2 and ALS cells and SCA2 mice. qRT-PCR analyses of STAU1 mRNA in SCA2 FBs and ALS FB with TDP-43G298S mutation (f) or SCA2 LBCs (g). h qRT-PCR analyses of cerebellar RNAs from ATXN2Q127 and BAC-Q72 mice compared to wild-type littermates (24 weeks of age; n = animals per group). Gene expression levels were normalized to Actb
