Huntington's Disease

Huntington’s disease is one of the timeless and dreaded diseases in the Western countries. Next to Cancer, the thought of this disease has affected a lot of people, mostly psychologically and emotionally.

While there is currently no cure for Huntington’s disease, promising research and new ideas on lifestyle factors offer new hope. Read further to learn the nature of Huntington’s disease. Who knows, probably the scientist in you may discover the cure for this disease.

Huntington's disease (often abbreviated "HD") is a progressive, inherited, degenerative brain disorder that produces physical, mental and emotional changes. Huntington's disease was known as Huntington's chorea, from the Greek for choreography, or dance. The term refers to the involuntary, jerky movements that can develop in later stages of the illness.

All humans have a protein called huntingtin in their nerve cells. Although scientists still need to determine huntingtin's exact function, it seems to play a critical role in the events that help nerve cells function effectively. Like majority of other proteins, huntingtin contains within it the amino acid glutamine. In people with HD, however, there are an excess number of glutamines in a particular segment of the protein. These excess glutamines come from having too many copies of the corresponding codon (the one that codes for glutamine) in the chemical code of DNA. The affected codon has the letters C-A-G. In the real sense, HD results from having too many copies of C-A-G in the DNA which codes for huntingtin protein. That's why HD is often referred to as a trinucleotide repeat disorder ("trinucleotide" being a fancy word for codon).

Here is a basic explanation of the biological basis of Huntington’s disease.

The specific actions of a protein are controlled by its unique 3-dimensional shape. The shape controls how the protein can "fit in" and interact with other parts of the cell. Its shape is determined by the type of amino acids that compose the protein, as well as by the specific order they are in. therefore, as with any well-engineered building, a successfully functioning protein starts with the "blueprints" (codons).

Huntington's disease was first described in medical literature in 1872 by Dr. George Huntington, a known physician from Long Island, New York. This disease affects men and women alike, occurring at a rate of about one in every 10,000 in most Western countries. The age of development of Huntington’s disease is normally between 30 and 50 years old, although there is also a form of HD that affects children and teenagers.

Although it's less common than some conditions characterized by similar symptoms, such as Parkinson's or Alzheimer's disease, approximately one person in 30,000 in the United States has Huntington's disease. Huntington's disease alters someone's ability to think, talk and move by destroying cells in the basal ganglia, the part of the brain that controls these capacities. Since caused by a gene mutation that leads to a toxic accumulation of protein in the brain, Huntington's is inherited from either one or both parents.

A child of a parent who carries the HD gene has a 50% chance of inheriting the abnormal gene. Pre-symptomatic testing can tell whether someone is likely to develop the disease. A child who gets the Huntington's gene will eventually develop the illness, although onset typically does not occur until ages 35-50 or later. If a child does not get the Huntington's gene, there is no risk of developing Huntington's disease or of passing it on, since Huntington's does not "skip" generations.

Around 30,000 people in the United States have Huntington's disease, which affects men and women equally across all ethnic and racial lines.