Wednesday, October 24, 2012

Possible therapeutic methods for ameliorating the Hutchinson–Guilford Progeria Syndrome Outlined

Thanks to a small international multidisciplinary team, we have a good set of hypotheses and an overview of potential therapeutic compounds for treating the HGPS. Some of these should be tested for the effects on progerin accumulation. 

The paper "Potential therapeutic approaches for modulating expression and accumulation of defective lamin A in laminopathies and age-related diseases, Zhavoronkov A, Smit-McBride Z, Guinan KJ, Litovchenko M, Moskalev A., J Mol Med (Berl). 2012 Oct 23"  in open access and is freely available for download as PDF.


Scientific understanding of the genetic components of aging has increased in recent years, with several genes being identified as playing roles in the aging process and, potentially, longevity. In particular, genes encoding components of the nuclear lamina in eukaryotes have been increasingly well characterized, owing in part to their clinical significance in age-related diseases. This review focuses on one such gene, which encodes lamin A, a key component of the nuclear lamina. Genetic variation in this gene can give rise to lethal, early-onset diseases known as laminopathies. Here, we analyze the literature and conduct computational analyses of lamin A signaling and intracellular interactions in order to examine potential mechanisms for altering or slowing down aberrant Lamin A expression and/or for restoring the ratio of normal to aberrant lamin A. The ultimate goal of such studies is to ameliorate or combat laminopathies and related diseases of aging, and we provide a discussion of current approaches in this review.