摘要: The nuclear lamina is the main architectural component of a eukaryotic nucleus. More than an inert scaffold, essential for maintaining proper organisation, epigenetic composition, transcriptional regulation and cell cycle progression. Mutations within lamin genes lead to wide range diseases known as laminopathies, among which striking premature aging disease Hutchinson–Gilford progeria syndrome (HGPS) most well known. Studies regarding cellular basis laminopathies have advanced our knowledge yielded remarkable insights into process normal human aging. Understanding molecular mechanisms responsible manifestations has also led development novel therapeutic strategies address lamina-related diseases. Ultimately, scientists clinicians seek provide treatment options laminopathy patients alleviate symptoms perhaps cure these in future. Key Concepts: Mutations encoding proteins laminopathies. The plays roles architecture, chromatin differentiation gene expression. Studies underlying advances understanding structure function. Progerin, mutant form A expressed syndrome, present individuals, albeit at low level. Recent noval suffering from HGPS. Keywords: nuclear lamina; lamins; laminopathies; muscular dystrophy; Hutchison–Gilford syndrome; epigenetics; farnesylation; morpholino
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