The A10S mutation caused the reduced levels of TRMU.
A, scheme for the multiple sequence alignment of the TRMU homologues. The position of A10S mutation is marked with an arrow. B, Western blotting analysis of six mutant and two control cell lines. 20 μg of total cellular proteins from various cell lines were electrophoresed through a denaturing polyacrylamide gel, electroblotted and hybridized with TRMU, MTO1, and NDUFB8, respectively, and with VDAC as a loading control. Quantifications of TRMU levels were determined as described elsewhere (15). The values for the mutant cell lines are expressed as percentages of the average values for the control cell lines. Cell lines harboring homozygous (−/−), heterozygous (+/−), or wild-type (+/+) TRMU mutations are indicated. Cell lines carrying the m.1555A→G (−) or wild type (+) are indicated.