Department of Medical Laboratory and Diagnostic Imaging, Faculty of Medicine, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary.
Department of Medical Laboratory and Diagnostic Imaging, Faculty of Medicine, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary.
Mutat Res. 2013 Oct-Dec;753(2):147-154. doi: 10.1016/j.mrrev.2013.08.002. Epub 2013 Sep 8.
Hydrogen peroxide was - and is still - considered toxic for a wide range of living organisms. Oxidative stress occurs when there is an excess of pro-oxidants over antioxidants and it has been implicated in several diseases. Catalase is involved in hydrogen peroxide catabolism and is important in defense against oxidative stress. Acatalasemia means the inherited near-total deficiency of catalase activity, usually in reference to red cell catalase. Acatalasemia was thought at first to be an asymptotic disorder. In the absence of catalase, neither the Japanese, or Hungarian acatalasemics nor acatalasemic mice had significantly increased blood glutathione peroxidase activity. In animal models, catalase deficient tissues show much slower rates of removal of extracellular hydrogen peroxide. In catalase knock-out mice, a decreased hydrogen peroxide removing capacity and increased reactive oxygen species formation were reported. Hydrogen peroxide may cause methemoglobinemia in patients with catalase deficiency. During anesthesia for a Japanese acatalasemic patient the disinfection with hydrogen peroxide solution caused severe methemoglobinemia. Patients with inherited catalase deficiency, who are treated with uric acid oxidase (rasburicase) may experience very high concentrations of hydrogen peroxide and may suffer from methemoglobinemia and hemolysis. The high (18.5%) prevalence of diabetes mellitus in inherited catalase deficient individuals and the earlier (10 years) manifestation of the disease may be attributed to the oxidative damage of oxidant sensitive, insulin producing pancreatic beta-cells. Ninety-seven of 114 acatalasemics had diseases related to oxidative stress and aging. The oxidative stress due to catalase deficiency could contribute to the manifestation of diabetes while for the other diseases it may be one of the factors in their causations. In summary, inherited catalase deficiency is associated with clinical features, pathologic laboratory test results, age and oxidative stress related disorders. Rather than considering it a benign condition, it should be considered as a complicating condition for aging and oxidative stress.
过氧化氢曾被认为——现在仍然被认为——对广泛的生物机体具有毒性。当促氧化剂超过抗氧化剂时，就会发生氧化应激，它与多种疾病有关。过氧化氢酶参与过氧化氢的分解代谢，在抵御氧化应激方面非常重要。无过氧化氢酶血症是指过氧化氢酶活性的近完全遗传性缺乏，通常是指红细胞过氧化氢酶。最初，无过氧化氢酶血症被认为是一种渐近性疾病。在没有过氧化氢酶的情况下，无论是日本型还是匈牙利型无过氧化氢酶血症患者，其血液谷胱甘肽过氧化物酶活性均无显著增加。在动物模型中，缺乏过氧化氢酶的组织显示出细胞外过氧化氢清除速度明显较慢。在过氧化氢酶敲除小鼠中，报道了过氧化氢去除能力降低和活性氧形成增加。过氧化氢酶缺乏的患者可能会导致高铁血红蛋白血症。在一名日本无过氧化氢酶血症患者的麻醉过程中，过氧化氢溶液的消毒导致了严重的高铁血红蛋白血症。接受尿酸氧化酶（拉布立酶）治疗的遗传性过氧化氢酶缺乏症患者可能会经历非常高浓度的过氧化氢，并可能患有高铁血红蛋白血症和溶血。遗传性过氧化氢酶缺乏症患者中糖尿病的高发（18.5%）和疾病的早期（10 年）表现可能归因于氧化剂敏感的胰岛素产生胰岛β细胞的氧化损伤。114 名无过氧化氢酶血症患者中有 97 名患有与氧化应激和衰老相关的疾病。由于过氧化氢酶缺乏引起的氧化应激可能导致糖尿病的发生，而对于其他疾病，它可能是其病因之一。总之，遗传性过氧化氢酶缺乏症与临床特征、实验室检查结果、年龄和氧化应激相关疾病有关。与其将其视为良性疾病，不如将其视为衰老和氧化应激的一种并发症。
