Genomic Instability and DNA Damage Responses in Progeria Arising from Defective Maturation of Prelamin A


Progeria syndromes have in common a premature aging phenotype and increased genome instability. The susceptibility to DNA damage arises from a compromised repair system, either in the repair proteins themselves or in the DNA damage response pathways. The most severe progerias stem from mutations affecting lamin A production, a filamentous protein of the… (More)