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Immunoblot analysis For detection of Gclc (Santa Cruz Biotechnology sc-390811), Pdha-1 (Abcam ab168379), DRP1 (Abcam ab184247), eIF4E (Abcam ab33766), p-eIF4E (Abcam ab76256), 4E-BP1 (Cell signaling 9452), p-4E-BP1 (Cell signaling 2855), DRP1 (Abcam ab184247), mTOR (Cell Signaling 2972), p-mTOR (Ser2448) (Cell Signaling 2971), p70 S6 (Cell Signaling 9202), p-p70 S6 (Thr421/Ser424) (Cell Signaling 9204), 15 10 6 cells were lysed with lysis buffer (CST 9803S) and protein/phosphatases inhibitors as per manufacturers protocol and blotted as described 44

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J Inherited Metab Dis (2010) 33:46977