You Fukka, Nicco Carole, Harakawa Yoshiaki, Yoshikawa Toshikazu, Inufusa Haruhiko
Division of Anti-Oxidant Research, Life Science Research Center, Gifu University, Yanagito 1-1, Gifu 501-1194, Japan.
Anti-Oxidant Research Laboratory, Louis Pasteur Center for Medical Research, Tanakamonzen-cho 103-5, Sa-kyo-ku, Kyoto 606-8225, Japan.
Int J Mol Sci. 2024 Mar 6;25(5):3064. doi: 10.3390/ijms25053064.
Systemic sclerosis (SSc) is an autoimmune disease characterized by systemic skin hardening, which combines Raynaud's phenomenon and other vascular disorders, skin and internal organ fibrosis, immune disorders, and a variety of other abnormalities. Symptoms vary widely among individuals, and personalized treatment is sought for each patient. Since there is no fundamental cure for SSc, it is designated as an intractable disease with patients receiving government subsidies for medical expenses in Japan. Oxidative stress (OS) has been reported to play an important role in the cause and symptoms of SSc. HOCl-induced SSc mouse models are known to exhibit skin and visceral fibrosis, vascular damage, and autoimmune-like symptoms observed in human SSc. The antioxidant combination Twendee X (TwX) is a dietary supplement consisting of vitamins, amino acids, and CoQ10. TwX has been proven to prevent dementia in humans with mild cognitive impairment and significantly improve cognitive impairment in an Alzheimer's disease mouse model by regulating OS through a strong antioxidant capacity that cannot be achieved with a single antioxidant ingredient. We evaluated the effectiveness of TwX on various symptoms of HOCl-induced SSc mice. TwX-treated HOCl-induced SSc mice showed significantly reduced lung and skin fibrosis compared to untreated HOCl-induced SSc mice. TwX also significantly reduced highly oxidized protein products (AOPP) in serum and suppressed Col-1 gene expression and activation of B cells involved in autoimmunity. These findings suggest that TwX has the potential to be a new antioxidant treatment for SSc without side effects.
系统性硬化症（SSc）是一种自身免疫性疾病，其特征为全身性皮肤硬化，伴有雷诺现象和其他血管疾病、皮肤及内脏器官纤维化、免疫紊乱以及多种其他异常情况。个体症状差异很大，针对每位患者寻求个性化治疗。由于SSc尚无根本治愈方法，在日本它被指定为疑难病症，患者可获得政府的医疗费用补贴。据报道，氧化应激（OS）在SSc的病因和症状中起重要作用。已知次氯酸诱导的SSc小鼠模型会出现人类SSc中观察到的皮肤和内脏纤维化、血管损伤以及自身免疫样症状。抗氧化剂组合Twendee X（TwX）是一种由维生素、氨基酸和辅酶Q10组成的膳食补充剂。TwX已被证明可预防轻度认知障碍患者的痴呆症，并通过其强大的抗氧化能力调节OS，从而在阿尔茨海默病小鼠模型中显著改善认知障碍，而单一抗氧化成分无法实现这一点。我们评估了TwX对次氯酸诱导的SSc小鼠各种症状的有效性。与未治疗的次氯酸诱导的SSc小鼠相比，经TwX治疗的次氯酸诱导的SSc小鼠的肺和皮肤纤维化明显减轻。TwX还显著降低了血清中高度氧化的蛋白质产物（AOPP），并抑制了参与自身免疫的Col - 1基因表达和B细胞活化。这些发现表明，TwX有可能成为一种无副作用的新型SSc抗氧化治疗方法。
