Biology 103
2003 First Paper
On Serendip

Progeria, an extremely rare disease caused by a slight genetic defect, victimizes every 1 in 4 million children. , (3). At the moment, there are twelve cases of Progeria in the US, and no more than one hundred have been reported around the world. While the child suffering from Progeria will appear to have no symptoms at birth, the tell tale signs of the fatal disease will begin to surface within a few months, (1). The common first symptom of a child who may be a Progerian is that the ends of their shoulder bones will be re-absorbed into their bodies. Soon, he or she will be underweight and undersize for his or her age. Hair loss and dental decay will follow. The disease slowly eliminates body fat. Eventually the Progerian will become afflicted by arthritis and take on the appearance of a person five to ten times their age, (6).On average, a Progerian will live to be thirteen. Usually their death will be due to a cardiovascular disease such as heart attack or stroke.

Over the past four years, a lot of progress has been made studying Progeria. Researchers have concluded the cause of this disease is most likely due to a "single letter misspelling in the genetic code on a single chromosome, which is a coiled strand of DNA within the cell". After examining twenty Progerians, eighteen were found to have the same genetic abnormality. The 19th case had a similar 'misspelling' in a nearby gene. The 20th case did not have "classic Progeria", (2).The gene which was found to be abnormal in eighteen of the cases, is responsible for making the protein called 'lamin A'. If this protein is defective, premature cell death occurs. This protein structures the inner layer of membrane surrounding the nucleus. Each Progerian examined had misshapen nuclear membranes in fifty percent of their cell. Persons examined without the disease have misshapen nuclear membranes in approximately one percent of their cells.

Since the likely cause of Progeria has been attributed to a glitch in genetic code, doctors and research scientists anticipate a cure. "It's not inconceivable that a basic treatment for Progeria could happen in the next two to three years," speculates Dr. W. Ted Brown, an expert in the study of Progeria, (2). Over $800,000 has been contributed to the Progeria Foundation. Because the disease is rare, most of the population was unaware that it even existed until articles were published in People Magazine, John Tackett (the oldest recorded survivor with Progeria at age fifteen) , (5) visited Maury Povich's daytime talkshow, and CNN discussed its tragic repercussions, (3). The supporters of the Progeria Foundation have been adamantly trying to make the public aware of its existence, in hopes to gain support and donations towards a cure. However, if a cure is on its way, will the children be merely survivors of a terribly unfortunate illness, or will they become milestones in a new theory about evolution?

A genetic mishap's ability to fast forward the process of aging is a scientific anomaly. Since Kindergarten, we have been taught of life's cycle. You are born, you become a toddler, child, teen, adult, have a midlife crisis, get old and wrinkley, and then you die. However, Progeria, with its one-gene-abnormality compresses all eight of those stages into thirteen years. What does this phenomenon say about the way we have regarded a natural human sequence?

People who lived in Roman times had a lifespan of twenty five to thirty years. The same lifespan was approximated for those living in London in the 18th century, , (4). In 1900 Americans had added thirty years onto their lives simply by introducing frequent pesticide use into the agricultural market and by advances made in the medical field. Simple conditions for health and sanitization have estimated that a WW1 baby in good physical shape can expect to live to be eighty years old. Does this increase in lifespan suggest that further progress will be made in the future to double or triple our current life expectancies? In the year 2300 will people live to be in their 200's?

Undeniably, societal adjustments would be made if the lifespan of the average person was extremely less or more. Our society practices laws that seem rational proportionally to the amount of time we live on this earth. But if we still lived to be only twenty five, would alcohol still be only legal past the age of twenty one? You get four years to drink beer in your 'twilight years' and then you die. The laws we practice and live by daily seem to imply an arbitrary moral code.

If the "misspelling" in genetic code associated with Progeria can be fixed by a doctor, allowing these children to slow down their process of aging, does that mean that a doctor could just as easily find a gene to tweek that would slow down the natural process of aging? Or is aging even natural at all?

Aging may very well be a puzzle in the midst of being solved. The evolution of man over time has, for the most part, provided a slightly changing pattern that runs well with its intention. However, diseases like Progeria seem to throw a monkey wrench into our understanding of human beings and time. While scientists may argue that the disease does not literally speed up the process of aging, and instead just replicates its signs - the visual evidence and anticipated cure debatably holds more weight. The negation of this rapid aging by simply correcting a typo in Progerians' genetic code would inadvertently mark a moment when technology caught up with evolution. This fix would allow human beings to manipulate evolution. Soon other alterations may be made to assess different problems which we had come to believe were 'natural'.

Is the evolution of man completely dependant on the evolution of knowledge and technology? By restraining ourselves to the confines of the theory of evolution, we may be neglecting to realize that there are exceptions to what we have come to know and believe is natural - even with evidence to the contrary before our eyes. Perhaps, if scientists became less skeptical of the seemingly irrational, progress would be made in areas of life never which were never even questioned. Maybe the expression, "it's not the way you look it's the way you feel" should be given a second thought. Little did we know that a burning contradiction to the theory of evolution could be found on the cover of a Hallmark birthday card.

References

2)Progeria Research Foundation, One of the few websites dedicated to the study of Progeria

3)Progeria Project, Provides articles and information about Progeria

4) Link from Berkely University Website, Interesting facts about lifespan

5) USA Today, Article about Progeria

6) CNN Link from Homepage, Detail the health issues involved with Progeria


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