ABCMdb

PMID: 15642351

Morita M, Takahashi I, Kanai M, Okafuji F, Iwashima M, Hayashi T, Watanabe S, Hamazaki T, Shimozawa N, Suzuki Y, Furuya H, Yamada T, Imanaka T
Baicalein 5,6,7-trimethyl ether, a flavonoid derivative, stimulates fatty acid beta-oxidation in skin fibroblasts of X-linked adrenoleukodystrophy.
FEBS Lett. 2005 Jan 17;579(2):409-14., [PubMed]

Sentences

No. Mutations Sentence Comment

34 ABCD1 p.Arg518Gln Cell culture Human skin fibroblasts (normal) and X-ALD fibroblasts (R518Q [13] and #163 [14]) were cultured in DMEM (ICN, Aurora, OH), containing 10% fetal bovine serum and maintained in a humidified atmosphere with 95% air and 5% CO2 at 37 °C. Login to comment 60 ABCD1 p.Arg518Gln Effect of various flavonoids on VLCFA b-oxidation in X-ALD fibroblasts The VLCFA b-oxidation activity in X-ALD fibroblasts (R518Q) was decreased by 70% as compared with normal fibroblasts (as shown in Fig. 1). Login to comment 71 ABCD1 p.Arg518Gln X-ALD fibroblasts (R518Q) were incubated in the absence or presence of 20 lM of various flavonoids for 72 h. Media were replaced every 24 h with the addition of fresh reagents. Login to comment 83 ABCD1 p.Arg518Gln Baicalein-tri-Me stimulates LCFA as well as VLCFA b-oxidation To avoid the possibility that the affect was restricted to certain X-ALD missense mutants, we attempted to elucidate the effect of the flavonoid on the activity in different mutant X-ALD fibroblasts; #163 (lacking ALD mRNA and protein, and immortalized by transformation with SV40) and R518Q (harboring the missense mutation of 1939G to A corresponding to the arginine at the 518 amino acid residue). Login to comment 84 ABCD1 p.Arg518Gln Baicalein- tri-Me stimulated VLCFA b-oxidation in both of the mutant X-ALD fibroblasts; #163 by 79% and R518Q by 117% (Table 1), suggesting that the effect was not restricted to a certain missense mutant. Login to comment 96 ABCD1 p.Arg518Gln ABCD1 p.Arg518Gln ABCD1 p.Arg518Gln ABCD1 p.Arg518Gln However, C22:0 was significantly decreased by the Table 1 Effect of baicalein-tri-Me on VLCFA b-oxidation in different X-ALD mutant fibroblasts Palmitic acid (C16:0) (pmol/h/ mg protein) Lignoceric acid (C24:0) (pmol/h/ mg protein) Healthy control None (DMSO) 225.7 ± 3.5 171.3 ± 7.4 Baicalein-tri-Me 357.3 ± 27.2 373.2 ± 1.3 X-ALD (R518Q) None (DMSO) 369.0 ± 90.2 71.1 ± 4.5 Baicalein-tri-Me 488.5 ± 42.2 154.1 ± 17.7 X-ALD (#163) None (DMSO) 296.9 ± 19.0 74.4 ± 1.4 Baicalein-tri-Me 589.9 ± 98.6 133.2 ± 9.5 Healthy control and X-ALD fibroblasts, R518Q and #163, were incubated with 30 lM of baicalein-tri-Me for three days. Login to comment 106 ABCD1 p.Arg518Gln X-ALD fibroblasts (R518Q) were treated with various concentrations of baicalein-tri-Me for three days (A) or cultured in the presence of baicalein-tri-Me at a concentration of 30 lM (B). Login to comment