Mate accumulation. . 107 proteins

Fibroblast cell lines from patients with aldehyde oxidase 1 (AOxl) and D-bifunctional protein (DBF) deficiency accumulated metabolic intermediates between 18 3n-3 and 24 6n-3 similar to control cells when incubated with [l- C] 18 3n-3 (Table 2). However, the rate of 22 6n-3 synthesis was <10% of control in these cell lines, indicating that these 2 peroxisomal fatty acid P-oxidation enzymes are involved in the retrocon-version of 24 6n-3 to 22 6n-3. The involvement of AOxl in the synthesis of 22 6n-3 was also demonstrated in vivo by Infante et al. (21) who detected less radiolabeled 22 6n-3 synthesis in AOxl knockout mouse livers compared with control litter-mates after the intraperitoneal injection of [U- C]18 3n-3. 

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