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KMID : 0391520150230020045
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Journal of the Korean Child Neurology Society
2015 Volume.23 No. 2 p.45 ~ p.50
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Valproic Acid Reduces Reactive Oxygen Species in Fibroblast of X-linked Adrenoleukodystrophy
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Kang Joon-Won
Quan Zhejiu
Kang Hoon-Chul
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Abstract
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Purpose: X-linked adrenoleukodystrophy (X-ALD) is a fatal, axonal demyelinating, neurodegenerative disease, and is caused by mutations the in ABCD1 (ATP-binding cassette transporter subfamily D member 1). Oxidative damage of proteins caused by very long chain fatty acid accumulating in X-ALD, is an early event in the neurodegenerative
cascade. We evaluated valproic acid (VPA) as a possible option for oxidative damage in X-ALD.
Method: We generated fibroblast of the childhood cerebral ALD from patient. We evaluated mRNA (ribonucleic acid) level of ABCD2 by real-time polymerase chain reaction, and reactive oxygen species (ROS) levels by flow cytometry.
Results: VPA increased expression of ABCD2 in both control and ALD fibroblast. ABCD2 gene mRNA expression was increased 1.76 fold in normal fibroblasts, and 2.22 fold in the X-ALD fibroblasts. ROS levels were decreased in VPA treated X-ALD fibroblast, especially in treated with 1 mM of VPA. ROS levels revealed 13.7 in control fibroblast, on the other
hand, 5.83 in X-ALD fibroblast treated with 1 mM of VPA.
Conclusion: We propose VPA as a promising novel therapeutic approach in oxidant damage that warrants further clinical investigation in X-ALD.
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KEYWORD
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Adrenoleukodystrophy, Valproic Acid, Reactive Oxygen Species
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