TABLE 2.
Quantification of the acyl-CoA species in the X-ALD fibroblasts
CCALD (n = 4) | AMN (n = 3) | Control (n = 5) | |||||||
Signals | Q1 (m/z) | Q3 (m/z) | Retention Time (min) | Amounta | Percent | Amounta | Percent | Amounta | Percent |
14:0-CoA | 978 | 471 | 0.97 | 18.3 | 1.6 | 13.9 | 1.9 | 15.5 | 2.3 |
15:0-CoA | 992 | 485 | 1.12 | 3.2 | 0.3 | 0.6 | 0.1 | 1.9 | 0.3 |
16:0-CoA | 1,006 | 499 | 1.34 | 102.0 | 8.9 | 48.6 | 6.5 | 71.4 | 10.5 |
16:1-CoA | 1,004 | 497 | 1.06 | 28.8 | 2.5 | 16.3 | 2.2 | 22.5 | 3.3 |
17:0-CoA | 1,020 | 513 | 1.62 | 25.5 | 2.2 | 18.2 | 2.4 | 23.8 | 3.5 |
17:1-CoA | 1,018 | 511 | 1.24 | 8.4 | 0.7 | 4.3 | 0.6 | 5.0 | 0.7 |
18:0-CoA | 1,034 | 527 | 2.07 | 31.8 | 2.8 | 15.6 | 2.1 | 21.4 | 3.2 |
18:1-CoA | 1,032 | 525 | 1.48 | 211.0 | 18.5 | 108.3 | 14.5 | 135.2 | 19.9 |
18:2-CoA | 1,030 | 523 | 1.21 | 23.9 | 2.1 | 15.4 | 2.1 | 18.5 | 2.7 |
18:3-CoA | 1,028 | 521 | 1.05 | 6.6b | 0.6 | 2.7 | 0.4 | 3.7 | 0.5 |
19:1-CoA | 1,046 | 539 | 1.79 | 3.3 | 0.3 | n.q. | 0.0 | 1.1 | 0.2 |
19:2-CoA | 1,044 | 537 | 1.40 | 1.3 | 0.1 | n.q. | 0.0 | 0.8 | 0.1 |
19:4-CoA | 1,040 | 533 | 1.30 | 9.4 | 0.8 | 8.8 | 1.2 | 12.5 | 1.8 |
20:0-CoA | 1,062 | 555 | 3.56 | 3.2 | 0.3 | 0.4 | 0.1 | 0.9 | 0.1 |
20:1-CoA | 1,060 | 553 | 2.25 | 9.9 | 0.9 | 5.5 | 0.7 | 5.9 | 0.9 |
20:2-CoA | 1,058 | 551 | 1.64 | 12.5c | 1.1 | 6.1 | 0.8 | 7.8 | 1.1 |
20:3-CoA | 1,056 | 549 | 1.36 | 74.7 | 6.5 | 43.8 | 5.9 | 54.9 | 8.1 |
20:4-CoA | 1,054 | 547 | 1.19 | 109.3 | 9.6 | 82.1 | 11.0 | 85.8 | 12.6 |
20:5-CoA | 1,052 | 545 | 1.03 | 18.5 | 1.6 | 7.5 | 1.0 | 12.1 | 1.8 |
21:0-CoA | 1,072 | 565 | 1.27 | n.q. | 0.0 | 0.6 | 0.1 | 1.0 | 0.1 |
21:3-CoA | 1,066 | 559 | 1.66 | 1.8b | 0.2 | 0.5 | 0.1 | 0.2 | 0.0 |
22:0-CoA | 1,090 | 583 | 5.28 | 5.1b | 0.4 | 4.2 | 0.6 | 1.5 | 0.2 |
22:1-CoA | 1,088 | 581 | 3.75 | 8.0 | 0.7 | 6.9 | 0.9 | 6.3 | 0.9 |
22:2-CoA | 1,086 | 579 | 2.67 | 1.4 | 0.1 | 0.4 | 0.0 | 0.6 | 0.1 |
22:3-CoA | 1,084 | 577 | 2.00 | 7.8 | 0.7 | 3.7 | 0.5 | 6.7 | 1.0 |
22:4-CoA | 1,082 | 575 | 1.62 | 50.0 | 4.4 | 18.0 | 2.4 | 31.6 | 4.6 |
22:5-CoA | 1,080 | 573 | 1.35 | 64.3c | 5.6 | 32.0 | 4.3 | 46.5 | 6.8 |
22:6-CoA | 1,078 | 571 | 1.20 | 22.2 | 1.9 | 11.5 | 1.5 | 16.8 | 2.5 |
24:0-CoA | 1,118 | 611 | 6.46 | 10.8b | 0.9 | 16.7 | 2.2 | 0.9 | 0.1 |
24:1-CoA | 1,116 | 609 | 5.32 | 33.6b | 2.9 | 33.4b | 4.5 | 12.9 | 1.9 |
24:2-CoA | 1,114 | 607 | 4.40 | 2.7 | 0.2 | 1.3 | 0.2 | 0.8 | 0.1 |
24:3-CoA | 1,112 | 605 | 3.21 | 3.7 | 0.3 | 1.8 | 0.2 | 3.2 | 0.5 |
24:4-CoA | 1,110 | 603 | 2.40 | 5.3 | 0.5 | 1.6b | 0.2 | 8.7 | 1.3 |
24:5-CoA | 1,108 | 601 | 1.94 | 7.5c | 0.7 | 3.6b | 0.5 | 6.5 | 1.0 |
24:6-CoA | 1,106 | 599 | 1.62 | 2.1 | 0.2 | 1.0 | 0.1 | 1.3 | 0.2 |
25:1-CoA | 1,130 | 623 | 5.92 | 15.2 | 1.3 | 16.6 | 2.2 | 9.2 | 1.4 |
26:0-CoA | 1,146 | 639 | 7.50 | 0.9 | 0.1 | 1.6 | 0.2 | n.q. | 0.0 |
26:1-CoA | 1,144 | 637 | 6.44 | 132.1b | 11.6 | 134.7d | 18.1 | 17.9 | 2.6 |
26:2-CoA | 1,142 | 635 | 5.79 | 15.3b | 1.3 | 11.2 | 1.5 | 3.6 | 0.5 |
26:5-CoA | 1,136 | 629 | 2.96 | 2.3 | 0.2 | n.q. | 0.0 | 1.6 | 0.2 |
27:1-CoA | 1,158 | 651 | 6.99 | 11.7d | 1.0 | 10.9d | 1.5 | 0.2 | 0.0 |
28:1-CoA | 1,172 | 665 | 7.49 | 26.0b | 2.3 | 24.1b | 3.2 | 0.1 | 0.0 |
28:2-CoA | 1,170 | 663 | 6.72 | 7.2b | 0.6 | 6.5 | 0.9 | 0.2 | 0.0 |
28:6-CoA | 1,162 | 655 | 6.07 | n.q. | 0.0 | 1.1 | 0.2 | 0.2 | 0.0 |
29:2-CoA | 1,184 | 677 | 6.52 | 1.6 | 0.1 | 1.3 | 0.2 | 0.5 | 0.1 |
Others | — | — | — | 3.2 | 0.3 | 0.9 | 0.1 | 0.3 | 0.0 |
Total | — | — | — | 1,143.4b | 100.0 | 744.3 | 100.0 | 680.7 | 100.0 |
VLCFA-CoA (DB = 0)e | 11.7 | 1.0 | 18.2 | 2.4 | 0.9 | 0.1 | |||
VLCFA-CoA (DB = 1)e | 218.6b | 19.1 | 219.8d | 29.5 | 40.5 | 5.9 | |||
VLCFA-CoA (DB = 2)e | 26.8b | 2.3 | 20.3b | 2.7 | 5.0 | 0.7 | |||
VLCFA-CoA (DB = 3)e | 3.7 | 0.3 | 1.8 | 0.2 | 3.2 | 0.5 | |||
VLCFA-CoA (DB = 4)e | 6.0 | 0.5 | 1.6b | 0.2 | 8.7 | 1.3 | |||
VLCFA-CoA (DB = 5)e | 9.8 | 0.9 | 3.6 | 0.5 | 8.1 | 1.2 | |||
VLCFA-CoA (DB = 6)e | 2.8 | 0.2 | 2.3 | 0.3 | 1.9 | 0.3 | |||
Total of VLCFA-CoA | 279.4b | 24.4 | 267.5d | 35.9 | 68.3 | 10.0 |
Expressed as picomoles per milligram of protein. The ratio of peak area for each acyl-CoA/D31-16:0-CoA was used to calculate the amount of each acyl-CoA species. n.q., below the quantitation range.
P < 0.05 versus control (Dunnett T3 post hoc test).
P < 0.05 versus AMN (Dunnett T3 post hoc test).
P < 0.01 versus control (Dunnett T3 post hoc test).
The total amount of each VLCFA-CoA species that contain very long-chain fatty acyl moieties with the number of double bonds (DBs) as indicated.