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

^aExpressed 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.

^bP < 0.05 versus control (Dunnett T3 post hoc test).

^cP < 0.05 versus AMN (Dunnett T3 post hoc test).

^dP < 0.01 versus control (Dunnett T3 post hoc test).

^eThe total amount of each VLCFA-CoA species that contain very long-chain fatty acyl moieties with the number of double bonds (DBs) as indicated.