Figure 1.

Alterations in the structure and stability of MT-ND1. (A) Electrostatic interactions in A52 of MT-ND1. Based on the cryo-EM structure of complex I from Homo sapiens (PDB entry: 5XTD), the hydrophobic residue A52 interacts with 48P, 55L, and 56F. (B) Western blot analysis. Cellular proteins (20 µg) from various cell lines were electrophoresed through a denaturing polyacrylamide gel, electroblotted, and hybridized with MT-ND1, MT-ND4, NDUFA3, NDUFA8, NDUFA13, AFG3L2, and CLPP antibodies, with GAPDH as a loading control. (C) Quantification of MT-ND1. Average relative values of MT-ND1 were normalized to the average values of VDAC1 in various cell lines. The calculations were based on three independent determinations in each cell line. The error bars indicate 2 SEM. P indicates significance based on Student's t-test of the differences between mutant and control cells.