Baicalein 5,6,7-trimethyl ether, a flavonoid derivative, stimulates fatty acid beta-oxidation in skin fibroblasts of X-linked adrenoleukodystrophy.
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.
Morita
Masashi
M
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
Takahashi
Ikuko
I
Kanai
Mariko
M
Okafuji
Fumito
F
Iwashima
Makoto
M
Hayashi
Toshimitsu
T
Watanabe
Shiro
S
Hamazaki
Tomohito
T
Shimozawa
Nobuyuki
N
Suzuki
Yasuyuki
Y
Furuya
Hirokazu
H
Yamada
Takeshi
T
Imanaka
Tsuneo
T
D000326:Adrenoleukodystrophy / Q000188:drug therapy / Q000378:metabolism*; D002478:Cells, Cultured; D002788:Cholesterol Esters / Q000378:metabolism; D005227:Fatty Acids / Q000032:analysis / Q000378:metabolism*; D005347:Fibroblasts / Q000737:chemistry / Q000187:drug effects / Q000378:metabolism; D044950:Flavanones / Q000494:pharmacology*; D005419:Flavonoids / Q000494:pharmacology / Q000627:therapeutic use; D006801:Humans; D010084:Oxidation-Reduction; D012867:Skin / Q000166:cytology
D002788:Cholesterol Esters; D005227:Fatty Acids; D044950:Flavanones; D005419:Flavonoids; C497523:baicalein 5,6,7-trimethyl ether