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• W2029177258 abstract "Lmna yields two major protein products in somatic cells, lamin C and prelamin A. Mature lamin A is produced from prelamin A by four posttranslational processing steps-farnesylation of a carboxyl-terminal cysteine, release of the last three amino acids of the protein, methylation of the farnesylcysteine, and the endoproteolytic release of the carboxyl-terminal 15 amino acids of the protein (including the farnesylcysteine methyl ester). Although the posttranslational processing of prelamin A has been conserved in vertebrate evolution, its physiologic significance remains unclear. Here we review recent studies in which we investigated prelamin A processing with Lmna knock-in mice that produce exclusively prelamin A (Lmna(PLAO)), mature lamin A (Lmna(LAO)) or nonfarnesylated prelamin A (Lmna(nPLAO)). We found that the synthesis of lamin C is dispensable in laboratory mice, that the direct production of mature lamin A (completely bypassing all prelamin A processing) causes no discernable pathology in mice, and that exclusive production of nonfarnesylated prelamin A leads to cardiomyopathy." @default.
• W2029177258 created "2016-06-24" @default.
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• W2029177258 date "2011-01-01" @default.
• W2029177258 modified "2023-09-27" @default.
• W2029177258 title "Investigating the purpose of prelamin A processing" @default.
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• W2029177258 doi "https://doi.org/10.4161/nucl.2.1.13723" @default.
• W2029177258 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/3104803" @default.
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