Mgme1

Total cell lysates were analyzed by SDS PAGE and immunoblotting. Primary antibodies used for western blotting were as follows: ATP5a, UQCR2, SDHB, COII, NDUFB8 (Abcam 110411, 1: 1000); 49KDa (home made, 1: 500); NDUFA9 (Abcam 14713, 1: 1000); H3 (Abcam 10799, 1: 10 000); Twinkle (home made, 1: 1000); TFAM (home made, 1: 5000); exoG (Proteintech 21523-1-AP, 1: 500); MGME1 (Sigma HPA040913, 1: 200); a-tubulin (Sigma T9026, 1: 5000); endoG (Abcam 9647, 1: 1000); Fen1 (home made, 1: 1000); PDH (MitoSciences MSP07, 1: 2500); Tomm20 (Abcam 56783, 1: 500); LC3B (Abcam 48394, 1: 2000); PINK1 (Abcam 75487, 1:1000). Secondary antibodies were as follows: Rabbit (HRP) anti-mouse (Sigma A9044, 1: 10 000) and Goat (HRP) anti-rabbit (Abliance BL2407, 1: 10 000). The detection of proteins was performed using Luminata forte western HRP Substrate (Millipore) or SuperSignal West Femto Maximum Sensitivity Substrate (Thermo Scientific). The relative abundance of specific proteins was evaluated by densitometric quantification of signal intensity, and analyzed using Quantity One image analysis software (BioRad).

Primary antibodies used for western blotting were as follows: MGME1 (Sigma HPA040913, 1 : 500 dilution), β-actin (Sigma A2228, 1 : 300 000), PolgA (Santa Cruz, 1 : 1000), mtSSB1 (kindly donated by Dr Kang) and TFAM (kindly donated by Dr Wiesner). Secondary antibodies used were as follows: goat anti-rabbit HRP (Promega W401B, 1 : 2000) and goat anti-mouse HRP (Promega W402B, 1 : 2000).