FIG. 1.

ALD mouse tissues have normal VLCFA (C_24:0) β-oxidation but accumulate VLCFA. (A) Total lipids were extracted, fractionated, and analyzed by gas chromatography as previously described (20). C_26:0/C_22:0 ratios are shown. These data were taken from Table 2 of reference 20 with permission. (B) VLCFA (C_24:0) β-oxidation activity (nanomoles per hour per milligram) was measured in wild-type (WT) and ALD mouse fibroblasts and tissues. Isolated peroxisomes were determined to be intact if most of the peroxisomal catalase activity was recovered in the peroxisomal pellet. Results are shown as means ± the standard deviations with the number of experiments performed in parentheses.