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HOPES For Kids (Non-Flash, Part 5) "Bryan's Dad Has Huntington's Disease"
An Illustrated Introduction to HD, For Kids & Families
Scenes 13-16
“Only a little, in the news and at school.” said Bryan. “I don’t really understand it. What does it do?”
“Well,” said the doctor, “DNA is chemical stuff in the body’s cells that carries information for sustaining life. Each cell in the body has DNA that contains many thousands of genes, but the problem in Huntington’s disease has to do with just one of these genes, one small piece of the DNA that is called the Huntington gene."
“Like every other gene, the Huntington gene contains many small sections called codons, each made of DNA. You can think of it like a chain: the entire Huntington gene is like a long chain and each codon is like a different link in the chain. In people who do not get Huntington’s disease, there are 35 or fewer identical links in a key part of the chain. But in people who do get Huntington’s disease, there are 40 or more of these identical links in the key part. Huntington’s disease results from having too many identical codons in the key part of the Huntington gene.”
“So the chain is too long?” asked Bryan. “Does it take up too much space or something?”
The doctor replied, “Well, not exactly. The system works like this: each gene makes another chemical called a protein and proteins are the things that do much of the work in cells. The Huntington gene makes a protein that we call huntingtin. In people who do not get Huntington’s disease, the huntingtin protein comes from a version of the Huntington gene with 35 or fewer identical codons in the key part. This huntingtin protein is able to do its normal work in cells. But in people who do get Huntington’s disease, the huntingtin protein comes from a version of the Huntington gene with 40 or more identical codons in the key part. This causes the huntingtin protein to have a different shape than normal and because of this altered shape, the protein cannot do its normal job.”
“After enough time, huntingtin proteins with the altered shape form into clumps or aggregations down inside cells, and we think these clumps clog up cells. We also think that the huntingtin proteins in these clumps may grab onto other proteins and keep them from doing their jobs in cells. So, these altered huntingtin proteins create a big problem, especially for nerve cells in the basal ganglia regions of the brain.”
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Last Modified: 10-24-04
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“Only a little, in the news and at school.” said Bryan. “I don’t really understand it. What does it do?”
“Like every other gene, the Huntington gene contains many small sections called codons, each made of DNA. You can think of it like a chain: the entire Huntington gene is like a long chain and each codon is like a different link in the chain. In people who do not get Huntington’s disease, there are 35 or fewer identical links in a key part of the chain. But in people who do get Huntington’s disease, there are 40 or more of these identical links in the key part. Huntington’s disease results from having too many identical codons in the key part of the Huntington gene.”
“So the chain is too long?” asked Bryan. “Does it take up too much space or something?”
“After enough time, huntingtin proteins with the altered shape form into clumps or aggregations down inside cells, and we think these clumps clog up cells. We also think that the huntingtin proteins in these clumps may grab onto other proteins and keep them from doing their jobs in cells. So, these altered huntingtin proteins create a big problem, especially for nerve cells in the basal ganglia regions of the brain.”
