Although there are drugs available to treat the symptoms of Huntington disease, there is no treatment yet to stop or reverse the disease. Researchers at MIT’s Center for Cancer Research have identified a compound called B2 that blocks one of the toxic effects of the misfolded huntingtin proteins in the brain. The researchers believe this compound might lead to a drug that could help stop the disease process.
Source:
Bodner, R., et al. (2006). Pharmacological promotion of inclusion formation: A therapeutic approach for Huntington’s and Parkinson’s diseases. Proceedings of the National Academy of Sciences, vol. 103, pp. 4246-4251.
Transplanted neural tissue improves HD symptoms temporarily
French researchers from Henri Mondor Hospital in Creteil transplanted fetal neural tissue into the brains of 5 individuals with Huntington disease, then followed their progress for 6 years. Three of the five showed improved motor and cognitive function for 4 years after receiving the tissue, but the effects were not permanent and began to decline after the fourth year. In the other two individuals, there was no response to the tissue transplant.
Source:
Bachoud-Lévi, A-C., et al. (2006). Effect of fetal neural transplants in patients with Huntington's disease 6 years after surgery: A long-term follow-up study. The Lancet Neurology, vol. 5, pp. 303-309.
Tetrabenazine reduces chorea
Individuals with Huntington disease experience chorea, the purposeless movement of arms and legs. Researchers at the University of Rochester School of Medicine and Dentistry studied the safety and efficacy of tetrabenazine for treating chorea for 12 weeks. Out of a group of 84 individuals with Huntington disease, 54 received tetrabenazine and 30 individuals received a placebo. Those receiving tetrabenazine reported greater reduction of their chorea than those receiving the placebo.
Source:
Huntington Study Group. (2006). Tetrabenazine as antichorea therapy in Huntington disease. Neurology, vol. 66, pp. 366-372.
Levels of mutant huntingtin influence the severity of HD in mice
Researchers are not yet clear on whether having two copies of the same allele of the Huntington disease gene (being homozygous) is associated with more severe disease. Scientists at the University of British Columbia examined mice with homozygous Huntington disease who had varying levels of mutant huntingtin. The results showed a clear relationship between levels of mutant huntingtin and disease, demonstrated by earlier age of onset and more rapid progression of symptoms. This supports the theory that more severe disease is associated with increased levels of mutant huntingtin as seen in homozygous Huntingon disease.
Source:
Graham, R.K., et al. (2006). Levels of mutant huntingtin influence the phenotypic severity of Huntington disease in YAC128 mouse models. Neurobiology of Disease, vol. 21, pp. 444-455.
Creatine treatment of Huntington disease
Researchers at the MassGeneral Institute for Neurodegenerative Disease and colleagues studied the effects of giving creatine to individuals with Huntington disease. They gave 64 individuals with the disease 8 grams per day of creatine for 16 weeks. The study showed this dose of creatine was safe and well tolerated, that blood and brain concentrations of creatine increased during the treatment, and that an indicator for oxidative injury to DNA was reduced by the creatine treatment.
Source:
Hersch, S.M., et al. (2006). Creatine in Huntington disease is safe, tolerable, bioavailable in brain and reduces serum 8OH2'dG. Neurology, vol. 66, pp. 250-252.
