Table 1.

Changes due to tafazzin deficiency in the contents (mol %) of the mouse organ and human lymphoblast phospholipids (PLs) relative to the normal content of CL (mol %) in the WT or healthy individual control ((TAZ-KDPL − WTPL)/WTCL or (BTHSPL − controlPL)/controlCL).a Numbers are shown for CL, choline (Cho), and ethanolamine (Etn) classes.

PhospholipidMouseHuman lymphoblast
HeartBrainLiverKidney
CL−0.69 ± 0.06+0.18 ± 0.67−0.01 ± 0.21−0.14 ± 0.11−0.73 ± 0.05
2-MLCL+0.38 ± 0.03+0.16 ± 0.02+0.17 ± 0.04+0.27 ± 0.02+0.64 ± 0.12
1-MLCL+0.13 ± 0.06+0.05 ± 0.05+0.11 ± 0.03+0.12 ± 0.02+0.23 ± 0.08
Diacyl PC (with plasmanylcholine)b+1.21 ± 0.54cccc
Plasmenylcholine−1.98 ± 0.65N.R.dN.R.dN.R.dN.R.d
Diacyl PE (with plasmanylethanolamine)b+0.59 ± 0.25+1.22 ± 1.81+3.76 ± 1.45+0.60 ± 0.66+3.37 ± 0.79
Plasmenylethanolamine+0.03 ± 0.08−4.56 ± 1.76−0.43 ± 0.24−0.32 ± 0.22−1.58 ± 0.96
  • a The average and error, shown as the SD, are obtained from three independent sets of biological samples (N = 3) for each of the WT and TAZ-KD mice, or healthy individuals and BTHS patients (Tables S1, S2, S3, and S4).

  • b The signal of plasmanyl glycerophospholipid as a minor component overlaps with the signal of the counterpart diacyl glycerophospholipid (Diagne et al, 1984; May et al, 1988; Kikuchi et al, 1999; Kimura et al, 2018).

  • c Content change in combination with that of plasmenylcholine is not discussed here because of a minor content of plasmenylcholine in the organ or blood cells (Diagne et al, 1984; May et al, 1988; Kikuchi et al, 1999; Kimura et al, 2018).

  • d Signal not resolved because of a minor content (Fig S1).