Deciphering the role of AP-1 transcription factor in cardiac involvement and inflammation of limb-girdle muscular dystrophy 2A

阐明AP-1转录因子在肢带型肌营养不良2A型心脏受累和炎症中的作用

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Abstract

Computational approaches overture profound insights into the complexities of nebulous diseases like Muscular Dystrophy including Limb-Girdle Muscular Dystrophy (LGMD). This research centers on LGMD 2A subtype (calapinopathy, OMIM #253600) and cardiac complications. Study aimed to investigate the misexpressed hub genes, consequences of disturbed pathways, and perception of ethnomedicines to manage the LGMD 2A. A comprehensive analysis the GSE11681 dataset revealed differentially expressed genes, which paved the way for constructing a protein-protein interaction network. Within this network, AP-1 emerged as the central transcription factor, receiving attention for its critical regulatory role. The study highlights a vital connection of AP-1 upregulation with pathways responsible for cardiac complications and inflammation in LGMD 2A patients. Study also provides a highlight of studied cases regarding cardiac involvement in LGMD 2A. To modulate the misexpression of FOS/AP1, Natural Product Activity and Spices Source Library containing ~ 30,926 natural compounds was screened. Previously proved compounds including Quercetin, Epigallocatechin gallate, Kaempferol, Paeoniflorin and Curcumin were also docked for comparative evaluation. From the Library, 4,5,6,7-tetrahydroxy-9,10-dioxoanthracene-2-carboxylic acid, Cimicifugic Acid C and D, Rhodocladonic Acid and Demethoxydeacetoxypseudolaric acid B emerged as the top candidates. Molecular Mechanics further analyzed complexes with Generalized Born and Surface Area solvation and molecular dynamic simulation. In conclusion, the study reveals the esoteric role of AP-1 as a contributing gene in LGMD 2A-associated cardiac complications and inflammation. The study concentrates on a specific cohort, with its findings potentially extrapolating to other populations sharing similar characteristics.

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