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• W4214645914 endingPage "2339" @default.
• W4214645914 startingPage "2339" @default.
• W4214645914 abstract "Myotonic dystrophy (DM) is a dominantly inherited multisystemic disorder affecting various organs, such as skeletal muscle, heart, the nervous system, and the eye. Myotonic dystrophy type 1 (DM1) and type 2 (DM2) are caused by expanded CTG and CCTG repeats, respectively. In both forms, the mutant transcripts containing expanded repeats aggregate as nuclear foci and sequester several RNA-binding proteins, resulting in alternative splicing dysregulation. Although certain alternative splicing events are linked to the clinical DM phenotypes, the molecular mechanisms underlying multiple DM symptoms remain unclear. Interestingly, multi-systemic DM manifestations, including muscle weakness, cognitive impairment, cataract, and frontal baldness, resemble premature aging. Furthermore, cellular senescence, a critical contributor to aging, is suggested to play a key role in DM cellular pathophysiology. In particular, several senescence inducers including telomere shortening, mitochondrial dysfunction, and oxidative stress and senescence biomarkers such as cell cycle inhibitors, senescence-associated secretory phenotype, chromatin reorganization, and microRNA have been implicated in DM pathogenesis. In this review, we focus on the clinical similarities between DM and aging, and summarize the involvement of cellular senescence in DM and the potential application of anti-aging DM therapies." @default.
• W4214645914 created "2022-03-02" @default.
• W4214645914 creator A5013419903 @default.
• W4214645914 creator A5057487885 @default.
• W4214645914 creator A5082836616 @default.
• W4214645914 date "2022-02-20" @default.
• W4214645914 modified "2023-09-26" @default.
• W4214645914 title "Cellular Senescence and Aging in Myotonic Dystrophy" @default.
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