Miyata Rie, Tanuma Naoyuki, Sakuma Hiroshi, Hayashi Masaharu
Department of Brain Development and Neural Regeneration, Tokyo Metropolitan Institute of Medical Science, Tokyo 156-8506, Japan; Department of Pediatrics, Tokyo-Kita Medical Center, Tokyo 171-0053, Japan.
Department of Brain Development and Neural Regeneration, Tokyo Metropolitan Institute of Medical Science, Tokyo 156-8506, Japan; Department of Pediatrics, Tokyo Metropolitan Fuchu Medical Center for the Disabled, Tokyo 183-8553, Japan.
Oxid Med Cell Longev. 2016;2016:5741517. doi: 10.1155/2016/5741517. Epub 2016 Apr 26.
Xeroderma pigmentosum group A (XPA) is a genetic disorder in DNA nucleotide excision repair (NER) with severe neurological disorders, in which oxidative stress and disturbed melatonin metabolism may be involved. Herein we confirmed the diurnal variation of melatonin metabolites, oxidative stress markers, and antioxidant power in urine of patients with XPA and age-matched controls, using enzyme-linked immunosorbent assay (ELISA). The peak of 6-sulfatoxymelatonin, a metabolite of melatonin, was seen at 6:00 in both the XPA patients and controls, though the peak value is lower, specifically in the younger age group of XPA patients. The older XPA patients demonstrated an increase in the urinary levels of 8-hydroxy-2'-deoxyguanosine and hexanoyl-lysine, a marker of oxidative DNA damage and lipid peroxidation, having a robust peak at 6:00 and 18:00, respectively. In addition, the urinary level of total antioxidant power was decreased in the older XPA patients. Recently, it is speculated that oxidative stress and antioxidant properties may have a diurnal variation, and the circadian rhythm is likely to influence the NER itself. We believe that the administration of melatonin has the possibility of ameliorating the augmented oxidative stress in neurodegeneration, especially in the older XPA patients, modulating the melatonin metabolism and the circadian rhythm.
A型着色性干皮病(XPA)是一种DNA核苷酸切除修复(NER)方面的遗传性疾病,伴有严重的神经障碍,其中可能涉及氧化应激和褪黑素代谢紊乱。在此,我们使用酶联免疫吸附测定(ELISA)法,证实了XPA患者及年龄匹配的对照组尿液中褪黑素代谢物、氧化应激标志物和抗氧化能力的昼夜变化。褪黑素的一种代谢物6 - 硫酸氧褪黑素的峰值在XPA患者和对照组中均出现在6:00,不过峰值较低,特别是在年轻的XPA患者组中。年龄较大的XPA患者的8 - 羟基 - 2'-脱氧鸟苷和己酰赖氨酸(氧化DNA损伤和脂质过氧化的标志物)的尿液水平升高,分别在6:00和18:00出现强劲峰值。此外,年龄较大的XPA患者的总抗氧化能力尿液水平降低。最近,据推测氧化应激和抗氧化特性可能存在昼夜变化,且昼夜节律可能会影响NER本身。我们认为,褪黑素的给药有可能改善神经退行性变中增强的氧化应激,特别是在年龄较大的XPA患者中,调节褪黑素代谢和昼夜节律。