volume 6, issue 9, P755-769 2014
DOI: 10.18632/aging.100680
View full text
Vittoria Cenni, Cristina Capanni, Elisabetta Mattioli, Marta Columbaro, Manfred Wehnert, Michela Ortolani, Milena Fini, Giuseppe Novelli, Jessika Bertacchini, Nadir M. Maraldi, Sandra Marmiroli, Maria Rosaria D'Apice, Sabino Prencipe, Stefano Squarzoni et al.

Abstract: Lamin A is a key component of the nuclear lamina produced through post-translational processing of its precursor known as prelamin A. LMNA mutations leading to farnesylated prelamin A accumulation are known to cause lipodystrophy, progeroid and developmental diseases, including Mandibuloacral dysplasia, a mild progeroid syndrome with partial lipodystrophy and altered bone turnover. Thus, degradation of prelamin A is expected to improve the disease phenotype. Here, we show different susceptibilities of prelamin…

expand abstract