Under the same conditions, lignoceric acid beta-oxidation was impaired in the above three peroxisomaldisease states.
2
Impairment of this function as occurs in peroxisomaldisease results in the accumulation of VLCFA and ULCFA.
3
The results reported suggest that various peroxisomaldisease states can be identified based on DHAP-AT and DHAP-synthase activities in skin fibroblasts of patients.