Towards Progeria Therapies

As noted briefly by Medical News Today, scientists are making progress towards the first therapies for the accelerated aging condition progeria (HGPS): "HGPS is caused by a mutation in the Lamin A gene (LMNA) that results in the synthesis of a mutant prelamin A (also called progerin). Progerin undergoes farnesylation but cannot be further processed to mature lamin A, a key structural component of the cell nucleus. In HGPS cells, progerin accumulates at the rim of the nucleus, causing misshapen nuclei. ... [researchers] suspected that protein farnesylation might be crucial for the aberrant targeting of progerin to the inner nuclear membrane and were able to show that blocking farnesylation with an inhibitor would prove therapeutic." Given that the same cellular problems are seen to a lesser but still significant degree in "normal" aging, it seems likely that any successful progeria therapy would also prove beneficial for the rest of us.


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