Huntington's disease is a degenerative disease of the brain that destroys specific brain cells. According to the Cleveland Clinic, people with the disease progress to develop uncontrollable movements and suffer mental deterioration and emotional problems. The symptoms of Huntington's disease usually begin in middle age. The disease can be more severe and progress more quickly when it starts in a young person. While medications can alleviate symptoms of Huntington's disease, a cure has not been found.
Inherited Gene
Huntington's disease is an inherited disease. People with Huntington's disease have a single gene that encodes the disease. According to the Massachusetts General Hospital Handbook of Internal Medicine, Huntington's disease is autosomal dominant, which means that only one copy of the disease gene, inherited from only one parent, is enough to cause the disease.
Cholesterol Accumulation
According to the Mayo Clinic, cholesterol is essential to the way healthy brain cells interact and send signals, and people with Huntington's disease have a defect in the way their brains use cholesterol. The gene that codes for Huntington's disease produces a protein that inhibits proper cholesterol metabolism in the brain. The defect in cholesterol accumulation may be at the root of the cognitive and motor problems in Huntington's disease patients.
Oxidative Stress
Highly reactive oxygen species, also known as free radicals, cause oxidative stress. That oxidative stress can cause cells to die. According to the book "Pathologic Basis of Disease" by Ramzi Cotran, oxidative stress causes a large amount of cell death in people with Huntington's disease. The destruction of brain cells may be one of the causes of Huntington's disease in those patients.
Impaired Energy Metabolism
According to the Massachusetts General Hospital Handbook of Internal Medicine, people with Huntington's disease have impairments of energy metabolism in their brain cells. Impaired energy metabolism in cells in regions of the brain called the basal ganglia and cortex cause many of the symptoms associated with Huntington's disease.
References
- Mayo Clinic: Huntington's disease
- Massachusetts General Hospital Handbook of Internal Medicine; Marc Sabatine; 2007
- Cleveland Clinic: Huntington's disease
- Pathologic Basis of Disease; Ramzi Cotran; 1999
