Baicalein 5,6,7-trimethyl ether, a flavonoid derivative, stimulates fatty acid beta-oxidation in skin fibroblasts of X-linked adrenoleukodystrophy |
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| Authors: | Morita Masashi Takahashi Ikuko Kanai Mariko Okafuji Fumito Iwashima Makoto Hayashi Toshimitsu Watanabe Shiro Hamazaki Tomohito Shimozawa Nobuyuki Suzuki Yasuyuki Furuya Hirokazu Yamada Takeshi Imanaka Tsuneo |
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| Institution: | Department of Biological Chemistry, Faculty of Pharmaceutical Science, Toyama Medical and Pharmaceutical University, 2630 Sugitani, Toyama 930-0194, Japan. masa@ms.toyama-mpu.ac.jp |
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| 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 beta-oxidation activity. Furthermore, the incorporation of 1-(14)C]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. |
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| Keywords: | ABC protein ATP-binding cassette protein ALDP adrenoleukodystrophy protein ALDRP ALDP-related protein baicalein-tri-Me baicalein 5 6 7-trimethyl ether LCFA long chain fatty acid X-ALD X-linked adrenoleukodystrophy PMP70 70-kDa peroxisomal membrane protein VLCFA very long chain fatty acid |
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