Progeria syndromes and ageing: what is the connection?
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.
Burtner
Christopher R
CR
Department of Biochemistry, University of Washington, Seattle, Washington 98195, USA.
Kennedy
Brian K
BK
D000375:Aging / Q000235:genetics / Q000502:physiology; D000818:Animals; D005075:Biological Evolution; D004249:DNA Damage; D004260:DNA Repair; D006801:Humans; D034904:Lamin Type A / Q000235:genetics; D008136:Longevity / Q000187:drug effects / Q000235:genetics / Q000502:physiology; D059630:Mesenchymal Stem Cells / Q000473:pathology / Q000502:physiology; D008954:Models, Biological; D011371:Progeria / Q000209:etiology* / Q000235:genetics / Q000503:physiopathology / Q000628:therapy; D015398:Signal Transduction; D020123:Sirolimus / Q000494:pharmacology; D013577:Syndrome
D034904:Lamin Type A; D020123:Sirolimus