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Antioxidant imbalance in the erythrocytes of Myotonic dystrophy Type 1 patients.
Archives of Biochemistry and Biophysics ( IF 3.8 ) Pub Date : 2019-12-20 , DOI: 10.1016/j.abb.2019.108230
Filiz Koc 1 , Gülüzar Atli 2 , Sule Yildiz Menziletoglu 2 , Sevgul Kose 3
Affiliation  

The most common form of muscular dystrophy is known as Myotonic dystrophy Type 1 (DM1) in adults. It was aimed to investigate the relationship between antioxidant imbalance and diaphragm thickness with pulmonary function test results in peripheral blood of Myotonic Dystrophy Type 1 patients. In the prospective study, 33 DM1 and 32 healthy control groups were taken after the ethics committee decision (2018-10529). Antioxidant defence system enzymes superoxide dismutase (SOD), catalase (CAT), glutathione peroxidase (GPX), glutathione reductase (GR), glutathione S-transferase (GST) and thiobarbituric acid reactive species (TBARS) levels were studied in blood samples. Also, muscular strength (MRC score), creatine kinase (CK) and diaphragm thicknesses were measured, and pulmonary function tests were performed. Among the studied parameters, TBARS levels and GPX, GR and GST activities in erythrocytes of DM1 patients showed a significant decrease in the range of 29-45% compared to the control group. MRC score, diaphragm thickness and inspiratory function test results at the end of inspiration and expiration were found lower though CK levels were higher in DM1 group. In the patient group, a positive correlation was found between antioxidant parameters (TBARS, CAT and GST) with diaphragm thicknesses and pulmonary function test though GPX showed a negative correlation with them. It was emphasized that the data obtained shows the harmful/pathogenic role of oxidative stress caused by free radicals in DM1, and also provide useful data for the treatment and processes of this disease.

中文翻译:

1型强直性肌营养不良患者红细胞中的抗氧化失衡。

成年人的肌肉营养不良最常见的形式是1型肌强直性营养不良(DM1)。目的探讨1型强直性肌营养不良患者外周血中抗氧化剂失衡和隔膜厚度与肺功能检查结果之间的关系。在前瞻性研究中,根据伦理委员会的决定(2018-10529),选择了33个DM1和32个健康对照组。在血液样本中研究了抗氧化防御系统酶超氧化物歧化酶(SOD),过氧化氢酶(CAT),谷胱甘肽过氧化物酶(GPX),谷胱甘肽还原酶(GR),谷胱甘肽S-转移酶(GST)和硫代巴比妥酸反应性物质(TBARS)的水平。此外,还测量了肌肉强度(MRC评分),肌酸激酶(CK)和隔膜厚度,并进行了肺功能测试。在研究的参数中,与对照组相比,DM1患者的TBARS水平以及GPX,GR和GST活性在29-45%的范围内显着降低。尽管DM1组的CK水平较高,但在吸气和呼气末时的MRC评分,隔膜厚度和吸气功能测试结果较低。在患者组中,尽管抗氧化剂参数(TBARS,CAT和GST)与隔膜厚度和肺功能测试之间呈正相关,但GPX与它们之间呈负相关。要强调的是,获得的数据显示了由DM1中的自由基引起的氧化应激的有害/致病作用,并且还为该疾病的治疗和过程提供了有用的数据。与对照组相比,DM1患者的红细胞GR和GST活性在29-45%的范围内显着下降。尽管DM1组的CK水平较高,但在吸气和呼气末时的MRC评分,隔膜厚度和吸气功能测试结果较低。在患者组中,尽管抗氧化剂参数(TBARS,CAT和GST)与隔膜厚度和肺功能测试之间呈正相关,但GPX与它们之间呈负相关。要强调的是,获得的数据显示了由DM1中的自由基引起的氧化应激的有害/致病作用,并且还为该疾病的治疗和过程提供了有用的数据。与对照组相比,DM1患者的红细胞GR和GST活性在29-45%的范围内显着降低。尽管DM1组的CK水平较高,但在吸气和呼气末时的MRC评分,隔膜厚度和吸气功能测试结果较低。在患者组中,尽管抗氧化剂参数(TBARS,CAT和GST)与隔膜厚度和肺功能测试之间呈正相关,但GPX与它们之间呈负相关。要强调的是,获得的数据显示了由DM1中的自由基引起的氧化应激的有害/致病作用,并且还为该疾病的治疗和过程提供了有用的数据。尽管DM1组的CK水平较高,但吸气和呼气末末的肌厚度和吸气功能测试结果较低。在患者组中,尽管抗氧化剂参数(TBARS,CAT和GST)与隔膜厚度和肺功能测试之间呈正相关,但GPX与它们之间呈负相关。要强调的是,所获得的数据显示了由DM1中的自由基引起的氧化应激的有害/致病作用,并且还为该疾病的治疗和过程提供了有用的数据。尽管DM1组的CK水平较高,但吸气和呼气末末的肌厚度和吸气功能测试结果较低。在患者组中,尽管抗氧化剂参数(TBARS,CAT和GST)与隔膜厚度和肺功能测试之间呈正相关,但GPX与它们之间呈负相关。要强调的是,获得的数据显示了由DM1中的自由基引起的氧化应激的有害/致病作用,并且还为该疾病的治疗和过程提供了有用的数据。CAT和GST)与diaphragm肌厚度和肺功能测试(尽管GPX呈负相关)。要强调的是,获得的数据显示了由DM1中的自由基引起的氧化应激的有害/致病作用,并且还为该疾病的治疗和过程提供了有用的数据。CAT和GST)与diaphragm肌厚度和肺功能测试(尽管GPX呈负相关)。要强调的是,所获得的数据显示了由DM1中的自由基引起的氧化应激的有害/致病作用,并且还为该疾病的治疗和过程提供了有用的数据。
更新日期:2019-12-20
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