Exploring the Biochemistry of Progeria

Is seems to be the case that the accelerated aging of progeria (HGPS) results from one minor component of "normal" aging run wild - the defects resulting from mutant lamin A. So it is plausible that therapies for progeria will have some value for the rest of us in due time. It is worth paying attention to the research: "Lamins are essential to maintain nuclear integrity and loss of lamin A/C results in increased cellular sensitivity to mechanical strain ... We found that skin fibroblasts from HGPS patients developed progressively stiffer nuclei ... fibroblasts from HGPS patients had decreased viability and increased apoptosis under repetitive mechanical strain, as well as attenuated wound healing, and these defects preceded changes in nuclear stiffness. Treating fibroblasts with farnesyltransferase inhibitors (FTI) restored nuclear stiffness in HGPS cells and accelerated the wound healing response in HGPS and healthy control cells [but] did not improve cellular sensitivity to mechanical strain. These data suggest that increased mechanical sensitivity in HGPS cells is unrelated to changes in nuclear stiffness and that increased biomechanical sensitivity could provide a potential mechanism for the progressive loss of vascular smooth muscle cells under physiological strain in HGPS patients."

Link: http://www.ncbi.nlm.nih.gov/pubmed/18331619

Comment Submission

Post a comment; thoughtful, considered opinions are valued. New comments can be edited for a few minutes following submission. Comments incorporating ad hominem attacks, advertising, and other forms of inappropriate behavior are likely to be deleted.

Note that there is a comment feed for those who like to keep up with conversations.