Huntington’s disease is a debilitating and tragic disease which affects one out of one hundred thousand people. Up until 2007, there was no known cure or effective treatment for Huntington’s chorea. In 2007, it was discovered that a drug used to
treat breast cancer could be used to treat Huntington’s chorea patients. This miracle drug is Trichostain C. Patients who have been treated by this drug show signs of slowed and decreased cell death in the brain and in some cases the cell death ceases entirely.Huntington’s disease or Huntington’s chorea (kor-ee’-yah) is an inherited autosomal dominant condition. HD is caused by a ‘trinucleotide repeat’ mutation which occurs on the 4th autosomal chromosome. The codon CAG (cytosine – adenine – guanine) is repeated 40 to 100 times where as in a healthy genome it is only repeated 26 times. This repetition causes the creation of 57 separate, defective genes. The variety of physical and emotional symptoms (uncontrollable jerking movements, extensive personality changes, loss of ability to talk fluently and walk normally) caused by HD is thought to be because of the copious amount of defective genes.
A healthy brain has neurons that are protected by the protein BDNF (Brain derived neurotropic factor) which encourages the growth and development of neurons and synapses. Huntington’s patients don’t produce enough BDNF, because their bodies produce the repressor, REST, which decreases the expression of this vital protein. Without BDNF, the neurons are vulnerable and susceptible to damage from other proteins produced by the defective genes.
The cancer treatment drug, Trichostain C, prevents the repressor, REST, from restricting the amount of BDNF. With this alteration the neurons will be protected from defective proteins and will continue to develop. Preventing cell death means that many of the advanced symptoms of HD will not arise. Trichostain C has already been through a several clinical trails so the impact of the drug has already been recognised and accounted for.
Trichostain C, could be a cure for Huntington’s disease. This would prevent HD patients from having to take a variety of Antipsychotics, Antidepressants, Tranquilizers, Mood stabilizers and Botulinum toxins to keep many of the symptoms of Huntington’s in check. Hopefully this treatment will be approved in the coming years.
References
Enderson, P. (2007, August 27), Huntington’s Treatment Blog, Cancer Drug Hope for Huntington’s Sufferers, Retrieved April 17, 2009, from http://huntingtonstreatment.com/
Swierzewski, S. (2000, January 2), Neurology Channel, Huntington’s disease: Treatment, Prognosis, Retrieved April 17, 2009, from http://www.neurologychannel.com/huntingtons/treatment.shtml
Elsley, C. (2007, August 26) Medical News Today, Breakthrough in Huntington’s research, Retrieved April 17, 2009, from http://www.medicalnewstoday.com/articles/80460.php
(2008, October 8)Genetic Interest Group, What is Huntington’s disease?, Retrieved April 19 2009, from
http://www.gig.org.uk/genesandyou_hd.htm
Collins, D. (1991, February 15), Genetics of Huntington’s disease. Genetics of Huntington’s disease, Retrieved April 19 2009, from http://www.kumc.edu/hospital/huntingtons/genetics.html
Interesting/ related links
Potential Cure using Pig Brain Cell Implants
http://blog.taragana.com/index.php/archive/human-study-of-potential-cure-for-huntingtons-disease-using-pigs-brain-cell-implants/
Research in Rats using Tetrabenazine
http://huntingtonstreatment.com/
