Progeroid laminopathies are characterized by the premature appearance of certain signs of physiological aging in a subset of tissues. They are caused by mutations in genes coding for A-type lamins or lamin binding proteins.
Here, scientists from SB2SM/I2BC review how different mutations causing progeroid laminopathies alter protein structure or protein-protein interactions and how these impact on mechanisms that protect cell viability and function. Targeting these molecular alterations in A-type lamins and associated proteins identified through structural biology studies could facilitate the design of therapeutic strategies to treat patients with rare but severe progeroid laminopathies.
![](/drf/joliot/PublishingImages/Actualites/Actualites/Actualites_institutionnelles/2020/Figure-Zinn-Progeria.png)
Uniform shape healthy nucleus (left); Nucleus with aberrant morphology in Progeria (right)
Image adapted from : The Cell Nucleus and Aging: Tantalizing Clues and Hopeful Promises. Scaffidi P, Gordon L, Misteli T. PLoS Biology 2005 3(11): e395. https://doi.org/10.1371/journal.pbio.0030395