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- W1964403054 abstract "We developed a specific method for direct determination of peroxisomal β-oxidation activity in cultured human skin fibroblasts. When control fibroblasts were incubated with N-(α-methylbenzyl)azelaamic acid (C9), a specific peroxisomal substrate, C5 and C7, the chain-shortened products, were detected with cell concentration and incubation time dependencies and no other products including C3 were detected. In glutaric aciduria type I and type II fibroblasts, the formation rates of C2 units liberated from C9 were almost similar to that in control cells. In contrast to these cell types, the fibroblasts from patient of Zellweger syndrome, in which peroxisomal β-oxidation was impaired, showed no conversion of C9 to C5 and C7. The lack of the C2 units liberation in Zellweger fibroblasts was not due to an impairment of mitochondrial β-oxidation and/or activation of C9 to C9-CoA derivative for subsequent β-oxidation reaction, but rather, appeared to be due to the specific defect of peroxisomal β-oxidation system. These results indicate that C9 is a useful substrate for the estimation of peroxisomal β-oxidation activity in cultured human skin fibroblasts." @default.
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- W1964403054 date "1988-07-01" @default.
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- W1964403054 title "Diagnosis of Zellweger syndrome by rectal biopsy: immunoblot of peroxisomal β-oxidation enzyme and activity of dihydroxyacetone phosphate acyltransferase in rectal mucosa" @default.
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- W1964403054 doi "https://doi.org/10.1016/0009-8981(88)90112-x" @default.
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