Abstract
The purpose of the present study is to identify bioactive compounds with potential for X-linked adrenoleukodystrophy (X-ALD) pharmacological therapy. Various plant natural products including flavonoids were tested for their ability to ameliorate the abnormality of very long chain fatty acid (VLCFA) metabolism in cultured skin-fibroblasts from X-ALD patients. Of the compounds tested, baicalein 5,6,7-trimethyl ether (baicalein-tri-Me) was found to significantly stimulate the VLCFA β-oxidation activity. Furthermore, the incorporation of [1- 14C]lignoceric acid into cholesteryl esters was markedly reduced towards the normal level and the VLCFA (C24:0 and C26:0) content was decreased. These results make baicalein-tri-Me a candidate for the therapeutic compound for X-ALD.
Original language | English |
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Pages (from-to) | 409-414 |
Number of pages | 6 |
Journal | FEBS Letters |
Volume | 579 |
Issue number | 2 |
DOIs | |
State | Published - 2005/01/17 |
Keywords
- ATP-binding cassette protein
- Adrenoleukodystrophy
- Baicalein
- Flavonoid
- Peroxisome
- Very long chain fatty acid
ASJC Scopus subject areas
- Biophysics
- Structural Biology
- Biochemistry
- Molecular Biology
- Genetics
- Cell Biology