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Decreased platelet membrane anisotropy in patients with adrenoleukodystrophy treated with erucic acid (22:1)‐rich triglycerides
Author(s) -
Stöckler S.,
Opper C.,
Greinacher A.,
Hunneman D. H.,
Korenke G. C.,
Unkrig C. J.,
Hanefeld F.
Publication year - 1997
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1023/a:1005309623452
Subject(s) - adrenoleukodystrophy , erucic acid , human genetics , membrane , medicine , endocrinology , biochemistry , chemistry , fatty acid , peroxisome , gene , receptor
Low platelet count and bleeding diathesis have been observed in patients with adrenoleukodystrophy (ALD) treated with erucic acid (22:1)‐rich triglycerides (‘Lorenzo's oil’). To investigate possible alterations of biophysical membrane properties, we measured platelet membrane anisotropy, which is inversely related to membrane fluidity, in 16 patients with and in 3 patients without treatment. In patients on treatment, platelet membrane anisotropy was significantly decreased. Additionally, we found increased platelet concentrations of 22:1 and compromised in vitro platelet aggregation response. The decrease of platelet membrane anisotropy is probably a main cause of bleeding diathesis. Long‐term haematological side‐effects must be considered in ALD patients treated with Lorenzo's oil.