Table 2.

Tissue total VLCFAs

	C22:0 %	C24:0 %	C26:0 %	C26:0/C22:0
Mouse tissue, total VLCFAs
Brain
Normal (n = 4)	0.701  ±  0.082	1.167  ±  0.155	0.020  ±  0.004	0.029  ±  0.005
X-ALD (n = 4)	0.902  ±  0.132	1.566  ±  0.123	0.108  ±  0.013	0.123  ±  0.028
Adrenal
Normal (n = 6)	0.124  ±  0.039	0.084  ±  0.024	0.009  ±  0.003	0.074  ±  0.010
X-ALD (n = 7)	0.093  ±  0.022	0.110  ±  0.033	0.024  ±  0.009	0.254  ±  0.068
Heart
Normal (n = 4)	0.379  ±  0.079	0.194  ±  0.030	0.006  ±  0.001	0.017  ±  0.001
X-ALD (n = 4)	0.412  ±  0.073	0.263  ±  0.031	0.025  ±  0.004	0.060  ±  0.006
Liver
Normal (n = 5)	0.643  ±  0.398	0.378  ±  0.121	0.004  ±  0.001	0.010  ±  0.007
X-ALD (n = 4)	0.673  ±  0.066	0.596  ±  0.100	0.014  ±  0.004	0.021  ±  0.005
Kidney
Normal (n = 4)	0.609  ±  0.020	1.090  ±  0.115	0.013  ±  0.002	0.021  ±  0.003
X-ALD (n = 4)	0.531  ±  0.028	1.492  ±  0.191	0.052  ±  0.008	0.098  ±  0.010
Human tissue, total VLCFAs
Brain
Normal (n = 19)	0.65  ±  0.09	3.69  ±  0.67	0.48  ±  0.09	0.74  ±  0.11
X-ALD*(n = 12)	0.46  ±  0.04	2.39  ±  0.46	1.09  ±  0.05	2.41  ±  1.25
X-ALD†(n = 10)	0.30  ±  0.09	1.99  ±  1.16	3.51  ±  2.61	10.76  ±  8.05
Liver
Normal (n = 4)	0.63  ±  0.27	0.71  ±  0.27	0.007  ±  0.004	0.009  ±  0.004
X-ALD (n = 7)	0.43  ±  0.13	0.94  ±  0.36	0.05  ±  0.02	0.12  ±  0.05

Measured values represent means ± SD. %, Percentage of total fatty acids. n, Total sample number measured. The differences of C26:0 % and C26:0/C22:0 values between normal and X-ALD are significant (P < 0.025) in all mouse and human tissues. 

^*Brain samples collected from mildly involved regions from X-ALD patients. 

^†Brain samples collected from severely demyelinated regions from X-ALD patients.