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Fig. 7 | Cell & Bioscience

Fig. 7

From: Pharmacological activation of ATF6 remodels the proteostasis network to rescue pathogenic GABAA receptors

Fig. 7

Mechanism of action of AA147 and AA263 on GABAA receptor proteostasis. AA147 and AA263 activate the ATF6 pathway to adapt the proteostasis network. They promote the folding of variant subunits by increasing BiP chaperone protein level and binding and enhance their forward trafficking by increasing LMAN1 protein level and binding. In addition, they attenuate the ERAD of variant subunits by inhibiting GRP94-HRD1/VCP-mediated degradation pathway. As a result, pharmacological activation of ATF6 increases the functional surface expression of variant GABAA receptors

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Cell & Bioscience

ISSN: 2045-3701

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