Therapeutic effects of docosahexaenoic acid ethyl ester in patients with generalized peroxisomal disorders.

@article{Martinez2000TherapeuticEO,
  title={Therapeutic effects of docosahexaenoic acid ethyl ester in patients with generalized peroxisomal disorders.},
  author={Maria Teresa Boyano Martinez and E. P{\'e}rez V{\'a}zquez and Mar{\'i}a Teresa Garc{\'i}a-Silva and Jesus Manzanares and Jos{\'e} Mar{\'i}a Bertr{\'a}n and Francesco Castello and Isabel Mougan},
  journal={The American journal of clinical nutrition},
  year={2000},
  volume={71 1 Suppl},
  pages={376S-85S}
}
Generalized peroxisomal disorders are severe congenital diseases that involve the central nervous system, leading to severe psychomotor retardation, retinopathy, liver disease, and early death. In these disorders, peroxisomes are not normally formed and their enzymes are deficient. Characteristically, plasmalogen synthesis and beta-oxidation of very-long-chain fatty acids (VLCFAs) are affected. We found that patients with generalized peroxisomal disorders have a profound brain deficiency of… CONTINUE READING
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