Hutchinson Gilford Progeria Syndrome

Putting the brakes on accelerated aging

Telomeres are to chromosomes what aglets are to shoelaces—protective caps that stop the tightly wound strings from unraveling. Every time a cell divides, its telomeres are trimmed, eventually becoming so short that the ...

Feb 11, 2016
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Researchers find new clues about aging

National Institutes of Health researchers have identified a new pathway that sets the clock for programmed aging in normal cells. The study provides insights about the interaction between a toxic protein called progerin and ...

Jun 13, 2011
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Aging disease in children sheds light on normal aging

(Medical Xpress) -- Aging of individual cells in the body leads to aging of the whole person. New evidence for this comes from studies of very rare children born with a genetic mutation that wrinkles, ages and kills them ...

Oct 24, 2011
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Progeria (also known as "Hutchinson–Gilford Progeria Syndrome", "Hutchinson–Gilford syndrome", and "Progeria syndrome") is an extremely rare genetic condition wherein symptoms resembling aspects of aging are manifested at an early age. The word progeria comes from the Greek words "pro" (πρό), meaning "before", and "géras" (γῆρας), meaning "old age". The disorder has very low incidences and occurs in an estimated 1 per 8 million live births. Those born with progeria typically live to their mid teens and early twenties. It is a genetic condition that occurs as a new mutation (de novo), and is rarely inherited. Although the term progeria applies strictly speaking to all diseases characterized by premature aging symptoms, and is often used as such, it is often applied specifically in reference to Hutchinson-Gilford Progeria Syndrome(HGPS).

Scientists are particularly interested in progeria because it might reveal clues about the normal process of aging. Progeria was first described in 1886 by Jonathan Hutchinson. It was also described independently in 1897 by Hastings Gilford. The condition was later named Hutchinson-Gilford Progeria Syndrome (HGPS).

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