Huntington's Disease Bibliography
Huntington's Disease Questions


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If one of your parents has Huntington's Disease, you have a 50% chance of inheriting the gene for the disease. If you inherit the gene from your parents, you will develop the disease at some point in your life, and will be able pass it onto your children. If you do not inherit the gene from your parents, the disease will not be able to be inherited.jpgpassed to your children.
As the gene is passed from one generation to the next, the number of repeats - called a CAG repeat expansion - get larger. The more times it repeats, the greater the chance of developing symptoms at a younger age. Therefore, as the disease is passed through generations in families, it becomes more evident in younger and younger ages.







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Mutations in the HTT gene cause Huntington’s Disease. The gene is located on Chromosome 4. The HTT gene holds the instructions to make the protein, Huntington, which are then distributed to different parts of the brain and different tissues in the human body.
Process:
1. Mutated forms chromosome4.jpgof Huntington’s disease include abnormally repeated segments of DNA. These repeated segments are called CAG trinucleotide (cytosine, adenine, and guanine).
2. These repeated segments cause protein syntheses (Huntington proteins) that contain large sections of the amino acid, glutamine.
3. The Huntington proteins are cut into pieces by enzymes in the cell. Molecules of glutamine stick out from the ends of the pieces of proteins.
  1. 4. The pieces then attach to other proteins to form clumps of proteins. These clumps of proteins have the potential to cause neuro-dysfunction (nerve cell). This leads to degeneration and the death of neurons in the group of nuclei in the part of the brain called the basal ganglia.



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As the brain cells in the basal ganglia start to decrease, the person diagnosed with HD starts to develop many symptoms. The symptoms may include the following: mood swings, depression, irritability, perssymptoms.jpgonality changes, and memory loss. In addition, they will also have trouble driving, learning new things, making decisions, and may start to have a hard time remembering facts. Involuntary and purposeless movements of legs, arms, and face (twitching) will also occur. This is also known as chorea, coming from the word choreography (dance-like movements). They will also begin to slur their words together, begin to lose weight, and have difficulty feeding themselves and swallowing. These symptoms occur gradually and later on in one’s life. Eventually, they leave the person unable to care for themselves and become dependent on others to care for them. Overall, the person diagnosed with Huntington’s Disease loses control of their mental and physical abilities.


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There is no cure for Huntington’s Disease, but medical advancements have been discovered that help reduce and slow down the symptoms catreatment.jpgused by this disease. Many people who have Huntington’s Disease show signs of depression, so medicines such as Desipramine, can be used to help depression, social withdrawal, and abnormal sleeping habits. Also, because people who have this disease often have movement problems, medications like Tetrabenazine are used to help reduce those symptoms. Since Huntington’s Disease affects the brain, it has been found that the transplantation of dopamine-containing cells has been attempted in a few places in the world. The results are unclear and therefore need to be further researched. Stem cell research has also been attempted to help reduce the damage that has been done to the brain.


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Forms of Huntington’s Disease

There are two forms of Huntington's disease. The most common is adult form of Huntington's disease. People with this form usually develop symptoms in their mid-30s and 40s. An earlier form of Huntington's disease accounts for a small number of cases and begins in childhood or adolescence.

Huntington's Disease Statistics Regarding Prevalence

  • Huntington's disease affects an estimated 3 to 7 per 100,000 people of European ancestry. The disorder appears to be less common in African Americans, Chinese, and Japanese people.
  • In the United States, around 30,000 people have Huntington's disease; estimates of its prevalence are about 1 in every 10,000 people.
  • At least 150,000 others have a 50 percent risk of developing Huntington's disease.
  • There is a 50 % chance for a child with one parent that has the disease to inherit it.

Interesting/Other Facts

  • American doctor George Huntington first described the disorder in 1872
  • In 1962 people with HD were thought to be possessed by the devil because they couldn't control their body movements and were condemned to death by fire
  • The more copied choromosomes one has, the more severe the disease gets
  • A person diagnosed does not die from HD directly, they die because it opens portals for infections, and weight loss effects that person's ability to fight infection
  • Weight loss is the more severe symptom
  • After symptoms begin to occur, the average person diagnosed with Huntington's Disease usually has 15 to 20 years left to live
  • A person diagnosed with this disease burns 5,000 calories a day