Fig 1: Mitochondrial GNMT decrease drives disruptions in Complex II activity. (A) WB analysis of GNMT and (B) global proteomic profile of freshly isolated liver mitochondria in the MCDD and MCDD-anti-miR-873-5p mice compared to control diet mice, changes are presented as ratios by intensity (colour scale bar). (C) BN-PAGE of 2% digitonin solubilized mitochondrial liver extracts, transferred to a membrane and immunoblotted for GNMT and SDHA. (D) Immunoprecipitation assay of mitochondrial liver extracts immunocaptured with CII and blotted for GNMT. (E) CII activity assay performed in the liver of the MCDD and (F) in Gnmt-KO mice. Data shown as average ± SEM. p < 0.05 *; p < 0.01 **.