A mixture of oleic, erucic and conjugated linoleic acids modulates cerebrospinal fluid inflammatory markers and improve somatosensorial evoked potential in X-linked adrenoleukodystrophy female carriers.

摘要: X−linked adrenoleukodystrophy is a rare inherited demyelinating disorder characterized by an abnormal accumulation of very long chain fatty acids, mainly hexacosanoic acid (26:0), due to mutation the gene encoding for peroxisomal membrane protein. The only available, and partially effective, therapeutic treatment consists dietary intake 4:1 mixture triolein trierucin, called Lorenzo’s oil (LO), targeted inhibit elongation docosanoic (22:0) 26:0. In this study we tested whether, besides inhibiting elongation, enhancement beta oxidation induced conjugated linoleic (CLA), will improve somatosensory evoked potentials modify inflammatory markers in females carriers. We enrolled five heterozygous women. They received LO (40 g/day) with CLA (5 2 months. efficacy was evaluated means plasma levels 26:0, 26:0/22:0 ratio, modification cerebrospinal fluid (CSF) potentials. Changes profile, particular incorporation, were also CSF plasma. results showed that promptly passes blood brain barrier able lower both 26:0 ratio improved potentials, which previously found unchanged or worsened alone, reduced IL-6 three out patients. Our data suggest synergic activity LO, enhancing beta-oxidation preventing formation, improves reduces neuroinflammation.