Progeria syndromes and ageing: what is the connection?

Authors: Burtner CR; Kennedy BK

Abstract: One of the many debated topics in ageing research is whether progeroid syndromes are really accelerated forms of human ageing. The answer requires a better understanding of the normal ageing process and the molecular pathology underlying these rare diseases. Exciting recent findings regarding a severe human progeria, Hutchinson-Gilford progeria syndrome, have implicated molecular changes that are also linked to normal ageing, such as genome instability, telomere attrition, premature senescence and defective stem cell homeostasis in disease development. These observations, coupled with genetic studies of longevity, lead to a hypothesis whereby progeria syndromes accelerate a subset of the pathological changes that together drive the normal ageing process.

Keywords: *Aging/genetics/physiology; Animals; Biological Evolution; DNA Damage; DNA Repair; Humans; Lamin Type A/genetics; Longevity/drug effects/genetics/physiology; Mesenchymal Stromal Cells/pathology/physiology; Models, Biological; Progeria/*etiology/genetics/physiopathology/therapy; Signal Transduction; Sirolimus/pharmacology; Syndrome
Journal: Nature reviews. Molecular cell biology
Volume: 11
Issue: 8
Pages: 567-78
Date: July 24, 2010
PMID: 20651707
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Burtner CR, Kennedy BK (2010) Progeria syndromes and ageing: what is the connection? Nature reviews. Molecular cell biology 11: 567-78.

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