14 posts tagged progeria

Zach Pickard 15 month old Zach Pickard is one of only 46 people in the world with progeria, an early aging disease.

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Seth Cook was a 13-year-old Washington boy who suffered from progeria, a disorder that causes accelerated aging He died last year after suffering a heart attack as a consequence of progeria (more…) progeria, seth, cook

Seth Cook, 1993-2007: Darrington teen with rare aging disorder dies Seth Cook, who lived in Darrington and captured hearts all over the world, died Monday at his home with his family around him. He was 13 years old and had recently suffered a heart attack as a consequence of progeria, a rare disorder

The soluble protein Klotho appears to be an anti-aging factor, since mice deficient in the Klotho gene show signs of premature aging. However, the validity of Klotho-/- mutants as a model of progeria is controversial: many of the pathological features of the mutant phenotype can be attributed to hypervitaminosis D, and can be reversed by eliminating vitamin D from the diet.

Unsurprisingly, but consistent with the idea that Werner’s syndrome is a good model for accelerated aging, WRN mutant mice are more prone than wildtype mice to develop obesity, insulin resistance, and diabetes when they are fed a diabetogenic diet. Moore et al.

Sometimes, biogerontologists get so enraptured by the molecular and cell-biological details of our studies that we forget what might be called the “human element” — the experience of an individual who is going through the process of aging.

Because every longevity-control gene eventually is eventually shown to interact with every other longevity-control gene, it is perhaps not surprising that SIRT1 deacetylates WRN, the protein whose gene is mutated in the devastating human progeria Werner’s Syndrome.

In a study published in the March issue of The Journal of Pediatrics, researchers found that decreased levels of HDL cholesterol, or “good” cholesterol, may contribute to premature heart disease in children with Hutchinson-Gilford Progeria Syndrome, or Progeria.