The structure of the cell nucleus is determined by the nuclear lamina, protein filaments that support the nuclear membrane and anchor the important components within the nucleus. Correct function of the lamina and its component parts are required in order for the cell to carry out vital functions such as nuclear DNA maintenance and repair, gene expression, and cell replication. In a cell with faulty nuclear lamina, the nucleus is misshapen and all these processes run awry. Such cells tend to become senescent in response to internal dysfunction, and cause damage to surrounding tissue via their inflammatory secretions if they are not then destroyed promptly by the immune system. Internal self-destruction mechanisms exist, but problems with gene expression may cause them to fail.
Regular readers will recall that this is the scenario in progeria, a condition with the appearance of accelerated aging. Progeria is caused by mutation in the lamin A gene that codes for a protein that is an important component the nuclear lamina. Progeria patients have dysfunctional, broken cells with misshapen cell nuclei, and as a consequence they die young of cardiovascular disease that is very similar to the
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