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4-Phenylbutyrate Attenuates the ER Stress Response and Cyclic AMP Accumulation in DYT1 Dystonia Cell Models

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Figure

Figure 1. Absence of torsinA or presence of torsinADdifferences in cAMP levels in torsinAPrimary mixed cortical and striatal neuronal cultures of individual E15 embryos from controls littermates (torsinA(torsinAViability Assay
Figure 2. Adenylate Cyclase (AC) activity is reduced in DYT1 dystonia patient fibroblast cells
Figure 3. 4-PBA alleviates ER stress in dystonia models. (A) Healthy control fibroblast cells lines (average of 3 lines: HF6, HF19 and GM02912)differences in sliced XBP1 (sXBP1) levels, an ER stress marker, upon 4-PBA treatments (2.5 and 5 mM) in healthy c
Figure 5. Model of torsinA modulation of cAMP. In healthy fibroblast cells (left panel), forskolin stimulates adenylate cyclase (AC) to synthesisaccumulation defects in DYT1 dystonia cell models by unoknow mechanism (s).cAMP, and there is no presence of En

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