Progeria and the Brittle Nucleus

(From EurekAlert!) Scientists continue to forge ahead in their understanding of the lamin biochemistry of progeria [HGPS], and the application of this knowledge to damage caused by "normal" aging. "The nucleus in all three trillion cells of the human body contains the DNA genome, which is wrapped with a stiff protein shell called the nuclear lamina. Children with HGPS have a mutation in one of the proteins of the lamina shell. ... the lamina shell in HGPS patients is stiffer than normal. However, stiffer isn't necessarily better. The stiffer lamina did protect the HGPS nucleus from some forces, but under excessive force the HGPS lamina was more brittle and eventually fractured. ... Once we understand what causes the lamina to stiffen, we can try to reverse or stop the problem. ... Our NIH collaborators have also found that the normal aged nuclei show the same structural changes as HGPS."



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