Abstract
In the Zellweger syndrome where peroxisomes are absent, extremely long fatty acids (24:0 and 26:0) accumulate in tissues suggesting that these fatty acids are normally β-oxidized in the peroxisomes. Previous studies with rat hepatocytes suggest that peroxisomes are also important in oxidation of C 22 unsaturated fatty acids. This study shows that cultured fibroblasts from normal human controls shorten [14- 14C]erucic acid (22:1( n−9)) to oleic acid (18:1( n—9)) efficiently while Zellweger fibroblasts are deficient in chain-shortening. [2- 14C]Adrenic acid (22:4( n−6)) is oxidized in control fibroblasts probably by chainshortening to arachidonic acid (20:4( n−6)). Only a little adrenic acid is oxidized in Zellweger fibroblasts. Linolenic acid (18:3( n−3)) is desaturated and chain-elongated in both control and Zellweger fibroblasts. The results support the view that peroxisomes play a normal physiological role in the shortening of C 22 unsaturated fatty acids and that this function is deficient in Zellweger fibroblasts.
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