1. Figure S1 The base peak chromatogram of the nanoLC–MS/MS analysis for the mitochondria proteins. Time(min) Relative Abundance 0 10 20 30 40 50 60 70 80 90 100 5 1020 30 152535 4050 60 455565 7080 90 758595

2. Figure S2 (A) Biological process and (B) cellular component analysis by gene oncology analysis of the total protein identified. A B mitochondrion organelle membrane mitochondrial membrane mitochondrial inner membrane membrane-enclosed lumen intracelluar organelle lumen mitochondrial lumen mitochondrial membrane part respiratory chain proton-transporting ATP synthase complex oxidation reduction generation of precursor metabolites and energy electron transport chain cellular respiration energy derivation by oxidation of organic compounds coenzyme metabolic process cofactor metabolic process nitrogen compound biosynthetic process aerobic respiration acetyl-CoA metabolic process

3. Figure S3 Profiling and of the quantitative analysis results by stable isotope labeling and nanoLC-MS/MS anlaysis.

4. ms 50 HR: 600 bpm 150250350450 45 ms P 50150250350 HR: 689 bpm ms 450 mV 1.1 0.7 0.4 -0.4 0 mV 1.1 0.7 0.4 -0.4 0 38 ms P UT-B +/+ UT-B -/- Figure S4 The II standard lead ECG of wild-type （ UT-B+/+ ） and UT-B null mice(UT-B-/-), arrowhead indicate P wave in ECG.