Searching for clues to premature aging.

In the late 1800s, Hutchinson reported two young boys with ‘congenital absence of hair and its appendages’. They, and an additional patient, were described further by Gilford, who proposed the term ‘progeria’ for this condition [1]. Hutchinson–Gilford Progeria syndrome (HGPS) is a rare developmental disorder affecting most of the organ systems in a manner that mimics, to some extent, features of natural aging but at a markedly accelerated rate [2]. In fact, HGPS has been considered as a prototype of premature aging syndromes, although the degree to which it truly recapitulates innate aging phenomena is still being debated. HGPS is thought to be a genetic disorder, yet the mode of inheritance, molecular basis and pathogenic mechanisms all remain elusive. To stimulate research on HGPS, in particular, and to extend our understanding of the aging processes, in general, the Massachusetts-based Progeria Research Foundation, jointly with the National Institutes of Health (NIH), organized an unprecedented international workshop on HGPS. The participants were representative of a spectrum of clinical expertise, and researchers from broad areas of molecular, cellular and developmental biology, as well as immunology, endocrinology, geriatrics and genetics, among others, presented their findings on this complex syndrome. The major focus was on the phenotypes of HGPS, with emphasis on the pathology of the extracellular matrix of connective tissue. It was hoped that clues from several organ systems would suggest promising avenues of research for understanding the mechanistic basis of this disease.