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.
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http://dx.doi.org/10.1038/s41598-022-09295-6 | DOI Listing |
J Ethnopharmacol
January 2025
Department of Gastroenterology, Suzhou Hospital of Anhui Medical University (Suzhou Municipal Hospital of Anhui province), NO.616 Bianyangsan Road, Suzhou, 234000, Anhui, China. Electronic address:
Ethnopharmacological Relevance: Inflammatory Bowel Disease (IBD), encompassing Ulcerative Colitis (UC) and Crohn's Disease (CD), stems from a multifaceted interaction of hereditary, immunological, ecological, and microbial elements. Current treatments have limitations, necessitating new therapeutic approaches.
Aim Of The Study: This study investigates the safeguarding impacts and fundamental processes of extracts of Gleditsia sinensis Lam.
Am J Chin Med
January 2025
Department of Neurology, The First Affiliated, Hospital of Anhui University of Traditional Chinese Medicine, Hefei, P. R. China.
Wilson's disease (WD) is a hereditary condition marked by abnormalities in copper metabolism, which precipitate a spectrum of neurological symptoms and cognitive impairments. Emerging research has highlighted ferroptosis (FPT) as a distinct type of programmed cell death, potentially linked to various cognitive dysfunctions. Nevertheless, the connection between FPT and cognitive impairment in Wilson's disease (WDCI) remains largely enigmatic.
View Article and Find Full Text PDFFront Mol Neurosci
September 2024
1st Department of Psychiatry, Psychotherapy and Early Intervention, Medical University of Lublin, Lublin, Poland.
Disturbances in pro/antioxidant balance emerge as a crucial element in bipolar disorder (BD). Some studies suggest that treatment effects on trace element concentration in BD. This study aimed to identify (a) the changes related to oxidative stress in BD and their relationship with trace elements engaged in pro/antioxidant homeostasis; (b) BD biomarkers using machine learning algorithm classification and regression tree (C&RT) analysis.
View Article and Find Full Text PDFTalanta
January 2025
Hunan Provincial Key Laboratory of Cytochemistry, School of Food and Bioengineering, School of Chemistry and Chemical Engineering, Changsha University of Science and Technology, Changsha, 410114, China. Electronic address:
The daily consumption of foods abundant in Glutathione (GSH) can be supplemented to maintain the homeostasis of GSH in human health and alleviate pathologies resulting from abnormal GSH levels. The fluorescence-based visual determination of GSH has gradually attracted the attention of researchers due to its robust performance and versatile implementation. However, the current GSH visual strategy primarily relies on variations in fluorescence intensity at a single emission wavelength, which poses challenges for naked-eye and portable readout, as well as distorted signals caused by complex matrix effects in real samples.
View Article and Find Full Text PDFFront Physiol
August 2024
Department of Hematology, The Second Xiangya Hospital, Molecular Biology Research Center, Center for Medical Genetics, School of Life Sciences, Hunan Province Key Laboratory of Basic and Applied Hematology, Central South University, Changsha, China.
Background: β-thalassemia (β-TH) is a hereditary hemolytic anemia that results in deficient hemoglobin (Hb) synthesis. It is characterized by ineffective erythropoiesis, anemia, splenomegaly, and systemic iron overload. Exploration new potential biomarkers and drug candidates is important to facilitate the prevention and treatment of β-TH.
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