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Laminopathies are something to keep an eye on nowadays, and this recent experimental determination only adds fuel to the fire. In this most recent work, it was shown that Lamin-A perturbations are apparently contributing to "normal human aging". It is already known that abnormalities in Lamin-A can lead to a type of Progeria known as Hutchinson-Gilford Progeria, but this most recent piece in Science indicates that similar dysfunction in this nuclear scaffold protein is seen in human aging.

Most of the mutations seen in Hutchinson-Gilford Progeria are de novo point mutations that introduce a "cryptic splice site" that produce truncated mRNAs that are not translated into "appropriate proteins". What these researchers found is that in normal aging, there begins to be the sporadic utilization of the same cryptic splice sites as is seen in Hutchinson-Gilford Progeria. In humans, this is thought to occur at exon 11, at which abberant splicing reduces the number of amino acids present at the carboxy terminus of the resultant peptide by 50 residues. This truncated peptide accumulates, and is apparently farnesylated, or tagged with a "fatty" precursor of cholesterol.

What is particularly interesting is that farnesyltransferase inhibitors have recently been shown to be effective in a mouse model of Hutchinson-Gilford Progeria. The novel findings being articulated in this study by a pair of researchers at the National Cancer Institute who produced this research would imply that there may be broader therapeutic applications of farnesyltransferase inhibitors than was thought previously.

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