Extended Data Fig. 1: PARL mediated STARD7 cleavage is required for maintaining CoQ levels.

a, Quantification of subunits of OXPHOS complexes I, II, III and IV and of the F₁F_O ATP synthase (complex V), detected by mass spectrometry of 5-week-old male wild-type and PARL^−/− brain tissues. Numbers of subunits detected for each complex are noted in the graph (n = 5 male mice). b, CoQ9 levels measured by mass spectrometry in brain and heart of 5-week-old male WT and PARL^−/− mice (n = 9 WT and n = 5 PARL^−/− animals). c, Immunoblot analysis of WT cells, PARL^−/− cells and PARL^−/− cells expressing PARL or PARL^S277A. d, CoQ9 abundance in HeLa cells lacking the indicated PARL substrate proteins (n = 10 WT, n = 4 STARD7^−/−, PARL^−/−, PGAM5^−/−, CLPB^−/−, n = 12 SMAC^−/−, n = 11 PINK1^−/− biologically independent samples). e, Immunoblot analysis of STARD7^−/− cells expressing mito-STARD7 or cyto-STARD7. f,g, CoQ9 levels measured in WT cells, STARD7^−/− cells, and in STARD7^−/− cells complemented with mito-STARD7 or cyto-STARD7 (n = 8 WT, STARD7^−/− and STARD7^−/− complemented with cyto-STARD7, n = 7 STARD7^−/− complemented with mito-STARD7 biologically independent samples) (f) or mito-STARD7^R189Q (g). h, Immunoblot analysis of PARL^−/− cells expressing mito-STARD7 or cyto-STARD7. i, CoQ9 abundance in WT cells, PARL^−/− cells and PARL^−/− cells expressing mito-STARD7 or cyto-STARD7. g,i, n = 5 biologically independent samples. c,e,h, Representative immunoblots of n = 2 independent experiments. a−b,f, The central band of each box is the 50% quantile, and the box defines the 25% (lower) and 75% (higher) quantile. The whiskers represent the minimum and maximum value in the data and outliers are indicated by a + sign (greater distance than 1.8 times inter quantile range away from the median). b,d,f,g,i, Data is represented by 95% confidence interval of the mean and the P values were calculated using a two-tailed Student’s t-test for unpaired comparisons where indicated.

Source data