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Progeria

Progeria is an extremely rare genetic condition which causes physical changes that resemble greatly accelerated aging in sufferers. The disease affects around 100 in 48 million newborns. Currently, there are approximately 35 known cases in the world. There is no known cure. more...

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Medicines

Most people with progeria die around 13 years of age. Progeria is of interest to scientists because the disease may reveal clues about factors involved in the process of aging, because it is an "accelerated aging" disease. But unlike most other "accelerated aging diseases" (like Werner's syndrome), progeria is not caused by defective DNA repair.

The condition was first identified in 1886 by Jonathan Hutchinson and Hastings Gilford. The condition was later named Hutchinson-Gilford Progeria syndrome; the name was derived from the Greek for "prematurely old". Around 100 cases have been definitely identified since then.

Cause

According to 'recent evidence, Progeria may be a de novo dominant trait. It develops during cell division in a newly conceived child or in the gametes of one of the parents. It is caused by mutations in a LMNA (Lamin A' protein) gene on chromosome 1.

Symptoms

Symptoms generally begin appearing around 18-24 months of age. The condition is distinguished by limited growth, alopecia and a characteristic appearance with small face and jaw and pinched nose. Later the condition causes wrinkled skin, atherosclerosis and cardiovascular problems. Mental development is affected. Individuals with the condition rarely live more than 16 years; the longest recorded life-span was 26 years. The development of symptoms is comparable to aging at a rate six to eight times faster than normal, although certain age-related conditions do not occur.

Read more at Wikipedia.org


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La pourriture noble
From Spectator, The, 10/14/00 by MacCann, Philip

THE WAY OF ALL FLESH:

A CELEBRATION OF

DECAY

by Midas Dekkers,

translated from the Dutch by Sherry

Marx-MacDonald

Harvil4 16.99, pp. 280

In a time of instant images and 15minute fame you might lament the death of culture. Yet 100 years ago Andre Gide declared European culture, high and low, `the destroyer of life'. Desperate to preserve and depict personal experience, Europeans were so bent on taking snapshots and purchasing souvenirs that it quite passed them by. At the ignoble heart of modern civilised culture was a horror of transience and our taboo of death. The French social historian Philippe Aries later argued that, while we have become so sanitising that we banish dying from sight altogether (to the hospital), we have lost the thrill of the moment.

It's no doubt with this romantic perspective, and its image of pre-capitalist innocence, that the Dutch biologist Midas Dekkers writes, reminding us that transience leading to deterioration and death is the very essence of life. We should reembrace it: in cellular decline since babyhood, we are decaying. Why can't decay be more sexy? This essay has none of the scientific analysis of P. B. Medawar attempting to understand the biology of senescence. Nor does it feel odd to be named after the autobiography of Samuel Butler, who consistently opposed the theory of natural selection. There is only a sprinkling of popular biology here. It's more a marvellously eccentric, if flippant, social comment defending the nuances and variegation of all things past their best. True connoisseurs relish the taste of fungi, the delicacy of rotting cheese, the melancholy of autumn, the beauty of ruins. To all these, Dekkers argues, age has brought venerability - `it's the difference between Mother Goose and Donald Duck'!

Why can't we just let things collapse in peace? `Nature conservation is so expensive,' he complains. `Many human hands are needed to make nature look untouched by human hand.' So conservationists, environmentalists, restorers, plastic surgeons, museum directors who keep up with fads - all appear as Puritans on a wrongheaded mission `to protect death from life', vainly demanding a retreat before the inexorable tide of algae, mosses, beetles, woodworm, not to mention waves, wind, rust, heat. When will these interferers realise `what's in store for us' - that `the greatest poet, the cruellest dictator, the most beautiful woman' will all be reduced to piles of dust. `Everything and everybody turns to dust.' There's more than a touch of Hamlet and adolescent morbidity here.

Dekkers (whose other books include a study of erotic liaisons between humans and animals, published in English as Dear Pet: On Bestiality) is direct, blasphemous and ambitious to be original in a way only the Dutch can be. One hundred and thirty black-and-white prints constitute an exotic catalogue of all things tasteless: a baby's severed hand in a bottle holding up an eye, slaughtered chickens, an execution in progress, a child with progeria.

With these pics we can celebrate the more venerable culture Dekkers loves, but which has also turned to dust. Now shallow youth rules with no experience of mixed emotions. Our whole society revolves around change, reducing experience to a burden. Bah! `Youth always wins.'

But hang on. This change is the very transience Dekkers claims to celebrate. His appeal to societies to accept decline seems in fact like a call to revive a more aesthetic sensibility and the book is one eloquent gesture of resistance to its decline. Perhaps it's this paradox which prevents Dekkers from taking his own argument too seriously. His anarchic, tongue-in-cheek humour is hilarious. For example: `Secretly guessing ages is the most popular pastime in trains and waiting rooms.' He roams into many areas of history and culture but stays clear of alchemy, which expressed the ancient and probably our most profound understanding of decay - the association with rebirth. (The grape transformed by fermentation into wine is 'a thousand times improved,' wrote Paracelsus, inventor of homeopathy, who called decay `the midwife of very great things'). Perhaps I'm carping. And who cares if Dekkers' argument is difficult to sustain? Maybe it's only the wail of an old person who finds himself invalidated, formed by one age to be judged by another. But for many who will feel similarly his book could be a precious relic of a more ambiguous age that seems to have passed away.

Copyright Spectator Oct 14, 2000
Provided by ProQuest Information and Learning Company. All rights Reserved

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