Abstract
The degradation of leukotrienes (LT) by β-oxidation from the ω-end proceeds in liver peroxisomes (J. Biol. Chem. 266:24763-72, 1991). Peroxisomal LT degradation was studied in man by analyses of endogenous LTs in urines from 9 patients with biochemically established peroxisomal deficiency disorders (PDD) and 9 age- and sex-matched healthy infants. LT metabolites were separated by reversed-phase HPLC and guantified by specific radioimmunoassays.
Results: Urinary LTE4 relative to creatinine (nmol/mol ± SD) increased from 59±23 in controls to 590±265 in patients, and N-acetyl-LTE4 from 16±13 to 212±169 nmol/mol, respectively (p<0.01). The β-oxidation product ω-carboxy-tetranor-LTE3 amounted to 48±33 nmol/mol in controls but was lacking in PDD. LTB4 was absent in normal urine but detectable in PDD (86±67 nmol/mol).
Conclusion: The impairment of degradation and inactivation of LT in PDD leads to an altered pattern of LT metabolites which is of diagnostic value and possibly of pathophysiological significance.
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Mayatepek, E., Jedlitschky, G., Schutgens, R. et al. IMPAIRED DEGRADATION OF LEUKOTRIENES IN PATIENTS WITH PEROXISOMAL DEFICIENCY DISORDERS. Pediatr Res 32, 627 (1992). https://doi.org/10.1203/00006450-199211000-00137
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DOI: https://doi.org/10.1203/00006450-199211000-00137