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| Good Health with Copper |
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| Genetic Abnormalities Linked to Copper |
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When proteins involved in the adsorption and distribution
of copper are not present in the human body, copper builds up in the
liver (Wilson's Disease) or the body fails to absorb copper (Menkes
Disease).
Fortunately, Wilson's Disease can now be managed by one of several
therapies that involve administering a chelator to the patient. The
chelator picks up and binds the copper and enables the patient to
excrete excess copper accumulated in the liver. The treatment allows
Wilson's sufferers to lead long and productive lives.
The protein produced by the Menkes gene is responsible for balancing
the intake and excretion of copper. A deficiency of this protein causes
copper to remain trapped in the lining of the small intestine, which
leads to copper deficiency in the body's organs. As a result, copper-dependent
enzymes cannot function properly in the body. The consequences are
coarse, brittle, depigmented hair and other neonatal problems, including
the inability to control body temperature, mental retardation, skeletal
defects, and abnormal connective tissue growth. For those with Menkes
Disease, ongoing research by ICA and other organizations is leading
to a greater understanding of the bodily mechanisms involved in the
disease and possible ways to treat the disorder.
Interestingly, the study of these diseases and the proteins produced
by the Wilson's and Menkes genes have enabled scientists around the
world to understand how our bodies use copper and why it is important
as an essential micronutrient. This knowledge is also leading scientists
towards possible cures for these diseases. One group of researchers
has already demonstrated in test tubes that damaged DNA in the cells
of a Menkes patient can be repaired. In time, the procedures needed
to accomplish this result in the human body will surely be found.
Idopathic Copper Toxicosis (ICT), the third genetic copper metabolic
disease, was recognized in the early twentieth century primarily in
the Tyrolean region of Austria and in the Pune region of India. ICT
appears to be vanishing as a result of greater genetic diversity within
these populations and educational programs to ensure that tinned cooking
utensils are used. Only occasional spontaneous cases of ICT arise
today. |
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