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Werner
syndrome is genetic recessive disorder. It is a type of progeria
disease that occurs in adults ages twenty to thirty. People who are
affected start to age rapidly beginning in their twenties and thirties
and look as though they are twenty or more years older that what they actually
are. Along with looking older patients develop other types of diseases
and disorders that occur with normal aging. "Werner's strikes about
three in every 1 million people worldwide, although it is slightly more
common in Japan" (MaughII). To the left is a picture of a Japanese
woman at age 14, and then pictured again at age 48. It is obvious
the disorder has made her look much older and frail than that of an average
48 year old woman.
Werner's patients physical characteristics are usually short with a stocky
trunk. They have hair that is thin and gray along with premature
balding. Their faces are prematurely aged as if they were 40 years older
than what they are and primarily has a beaked nose. The picture to
the left is of a man who has many of these characterizes. The second
picture is of his foot which has developed a another type of disorder related
to aging called scaradoma. It is on of the many types of diseases and disorders
that develop with Werner because of the premature ageing. Below is
a list of common disorders that may develop.

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Sclerodermais
a autoimmune disease of connective tissue where there is an over production
of collagen fibers, also classified as systemic sclerosis. There may be
visible signs of the skin. It affects the face and distal extremities with
subcutaneous calcification and ulceration. The invisible part of scleroderma
affects the internal organs such as the esophagus, lungs, kidneys, heart,
blood vessels, muscles and joints. |
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Arteriosclerosis
is the hardening and thickening of the walls of the arteries which causes
narrowing of the blood vessels resulting in decrease of blood flow in the
arteries. |
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Diabetes
mellitus is a metabolic disease where carbohydrates are not being
broken down because of the lack of insulin secreted by the pancreas. This
disease is a type of endocrine disorder. There are many other types of |
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Endocrine
disorders that people with Werner may have. Endocrine glands secrete
hormones into the blood stream and have an affect on the body's systems.
A lack of an endocrine hormone causes an endocrine disorder. Unusual cancers
such as |
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Thyroid
carcinoma is an unuasal cancer that may develop in people who have
Werner Syndrome. It is caused by the high production of calcitonin. |
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Cataract
disorders are where the lens of the eye becomes partly or completely
obscure. |
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Retinal
disorders are muscular degeneration or retinal dystrophy. |

GENETIC
INHERITANCE OF THESE DISEASES
CELLULAR
MECHANISMS
THERAPY

Works
Cited
Maugh
II, Thomas H. "Science: Discovery could lead to new treatments for heart
disease, cancer, and osteoporosis, experts say. (Human Gene That Causes
Aging Symptoms Identified) (Part A)." Info Trac Web: Custom Newspapers.
<CJ57146224>.
King,
Warren. "Gene that affects aging is isolated in the patients with rare
disease." Info Trac Web: Custom Newspapers. <CJ65393377>.
References:
OMIM
Entry 277700. National Certer for Biotechnology Information/OMIM. Online
Medelian Inheritance in Man. 2 February 2001. http://www3.ncbi.nlm.nih.gov/htbin-post/Omim/dismim?277700
"Werner
Syndrome (WS)." Clinical Molecular Genetics Society Website. 2 February
2001. http://www.ich.ucl.ac.uk/cmgs/werngene.htm
Other
links
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http://www.ncbi.nlm.nih.gov/cgi-bin/SCIENCE96/gene?WRN
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http://www.science.uwaterloo.ca/~bepillon/werner.htm
Credits
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Picture of a Japanese woman and the outcome
of the disease at age 48. (Pennisi, 1996) and to William and Wilkens
Publishing Co. From the What is Werner Syndrome web page.
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Picture of a patient with Werner Syndrome
(Adoue, 1999). From the What is Werner Syndrome web page.
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