School of Interdisciplinary Studies, Jamia Hamdard, New Delhi, India.
Department of Pharmaceutical Chemistry, School of Pharmaceutical Education and Research, Jamia Hamdard, New Delhi, 110062, India.
Sci Rep. 2022 Mar 31;12(1):5474. doi: 10.1038/s41598-022-09295-6.
Hereditary glutathione reductase deficiency, caused by mutations of the GSR gene, is an autosomal recessive disorder characterized by decreased glutathione disulfide (GSSG) reduction activity and increased thermal instability. This study implemented computational analysis to screen the most likely mutation that might be associated with hereditary glutathione reductase deficiency and other diseases. Using ten online computational tools, the study revealed four nsSNPs among the 17 nsSNPs identified as most deleterious and disease associated. Structural analyses and evolutionary confirmation study of native and mutant GSR proteins using the HOPE project and ConSruf. HOPE revealed more flexibility in the native GSR structure than in the mutant structure. The mutation in GSR might be responsible for changes in the structural conformation and function of the GSR protein and might also play a significant role in inducing hereditary glutathione reductase deficiency. LD and haplotype studies of the gene revealed that the identified variations rs2978663 and rs8190955 may be responsible for obstructive heart defects (OHDs) and hereditary anemia, respectively. These interethnic differences in the frequencies of SNPs and haplotypes might help explain the unpredictability that has been reported in association studies and can contribute to predicting the pharmacokinetics and pharmacodynamics of drugs that make use of GSR.
遗传性谷胱甘肽还原酶缺乏症是一种常染色体隐性遗传病,由 GSR 基因突变引起,其特征为谷胱甘肽二硫化物(GSSG)还原活性降低和热稳定性增加。本研究采用计算分析方法筛选与遗传性谷胱甘肽还原酶缺乏症和其他疾病相关的最可能突变。使用十种在线计算工具,研究发现 17 个非编码单核苷酸多态性(nsSNP)中有 4 个被认为最具破坏性和与疾病相关。使用 HOPE 项目和 ConSruf 对天然和突变 GSR 蛋白进行结构分析和进化确认研究,结果表明天然 GSR 结构比突变体结构更具灵活性。GSR 中的突变可能导致 GSR 蛋白结构构象和功能的变化,并可能在诱导遗传性谷胱甘肽还原酶缺乏症中发挥重要作用。对该基因的 LD 和单倍型研究表明,鉴定出的 rs2978663 和 rs8190955 变异可能分别与阻塞性心脏缺陷(OHD)和遗传性贫血有关。这些 SNP 和单倍型在不同种族中的频率差异可能有助于解释关联研究中报道的不可预测性,并有助于预测利用 GSR 的药物的药代动力学和药效学。