DescriptionBiogenesis of lamin A in normal cells and the failure to generate mature lamin A in HGPS.jpg
English: Biogenesis of lamin A in normal cells and the failure to generate mature lamin A in HGPS. In the setting of ZMPSTE24 deficiency, the final step of lamin processing does not occur, resulting in an accumulation of farnesyl-prelamin A. In HGPS, a 50-amino acid deletion in prelamin A (amino acids 607–656) removes the site for the second endoproteolytic cleavage. Consequently, no mature lamin A is formed, and a farnesylated mutant prelamin A (progerin) accumulates in cells. Coutinho et al. Immunity & Ageing 2009 6:4 doi:10.1186/1742-4933-6-4
Русский: Биогенез ламина A в нормальных клетках и сбой генерации зрелой формы ламина A при синдроме прогерии Хатчинсона-Гилфорда.
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