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Corruption of DNA end-joining in mammalian chromosomes by progerin expression
DNA Repair, 2023Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare genetic condition characterized by features of accelerated aging and a life expectancy of about 14 years. HGPS is commonly caused by a point mutation in the LMNA gene which codes for lamin A, an essential component of the nuclear lamina.
Liza A, Joudeh +9 more
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Nucleocytoplasmic transport in cells with progerin-induced defective nuclear lamina
Biophysical Chemistry, 2017Recent data indicate that nuclear lamina (NL) plays a relevant role in many fundamental cellular functions. The peculiar role of NL in cells is dramatically demonstrated by the Hutchinson-Gilford progeria syndrome (HGPS), an inherited laminopathy that causes premature, rapid aging shortly after birth.
Ferri G, Storti B, Bizzarri R
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Increased progerin expression associated with unusualLMNAmutations causes severe progeroid syndromes
Human Mutation, 2007Hutchinson-Gilford progeria syndrome (HGPS) is a rare precocious aging syndrome caused by mutations in LMNA that lead to synthesis of a mutant form of prelamin A, generally called progerin, that cannot be processed to mature lamin A. Most HGPS patients have a recurrent heterozygous de novo mutation in exon 11 of LMNA, c.1824C>T/p.G608G; this synonymous
Moulson, Casey L. +8 more
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Progerin - novi uzrok starenja stanica
2011Prema današnjim spoznajama fiziološko se starenje stanice objašnjava progresivnim skraćivanjem telomernih dijelova DNA. U normalnim somatskim stanicama ovog enzima ima u minimalnim količinama pa su postupni gubitak telomera i posljedično starenje neizbježni. Nefiziološko starenje stanice mogu pokrenuti razni vanstanični ali i stanični poticaji, kao što
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Nuclear membrane deformation via progerin clustering
Biophysical Journal, 2023openaire +1 more source
The Role of Progerin in Artificially Ageing Stem Cells
Stem Cell Reviews and ReportsRachel Brown, Mubeen Goolam
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Dissecting early neuronal ageing features in Progerin-overexpressing organoids
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