Arches. Photo by Daniel Chia
HOPES: Huntington's Outreach Project for Education, at Stanford
Jul
16
2011

Pridopidine (Huntexil, ACR-16)

Drug Summary: Pridopidine, also known as Huntexil or ACR-16, is a dopamine stabilizer intended to improve voluntary movements and reduce chorea. Initial clinical trials – the MermaiHD and HART studies – show promising results, but drug regulation agencies have requested another trial before pridopidine can be sold to the general public. control relevant M. Hedlin, [...]

Jun
30
2011

About Disease Mechanisms

Welcome to the "Drugs and Supplements" section of the HOPES website! Articles within this section will frequently use the terms "treatment" and "cure." Please note, though, that the word "treatment" must not be confused with the word "cure," for there is currently no medical cure for Huntington's Disease (HD).

Jun
30
2011

About Huntington’s Disease and Serotonin

Serotonin (also known as 5-HT) is a neurotransmitter used to communicate important information between nerve cells. Serotonin is sometimes referred to as the “feel good” neurotransmitter owing to its association with elevated mood levels. It also has many other functions in the central nervous system including roles in sleep, depression, memory, pain, and aggression. Recent [...]

Jun
29
2011

About Free Radical Damage

Aside from impaired energy production, damage to the mitochondria leads also to increased production of toxic molecules called free radicals. Compounds called antioxidants act as free radical scavengers by initiating reactions that make free radicals non-toxic to cells. Evidence indicates that damage by free radicals is a contributing factor to the pathology of HD. Consequently, [...]

Jun
26
2011

About Protein Aggregation

The altered huntingtin protein responsible for HD has been shown to contain many more molecules of the amino acid glutamine than regular huntingtin. This abundance of glutamine is due to repetitive copies of the CAG codon in the Huntington gene. The extended glutamine tracts of these proteins have affinity for one another, and tend to [...]

Jun
26
2011

About Changes in Gene Transcription

Compounds that have the ability to regulate the transcription of specific sets of genes are being proposed as possible candidates for treating HD. Some of these compounds may be able to regulate genes to produce proteins that could delay various HD symptoms. For example, some of the drugs could induce the transcription of genes responsible for the production of proteins with anti-inflammatory effects.

Jun
26
2011

About Abnormalities in Energy Metabolism

The mutant huntingtin protein has been found to disrupt cellular metabolism, the process by which cells make energy. It interacts with key proteins needed to produce energy and causes damage to mitochondria, the ‘energy factory’ of the cell. Mitochondria produce energy in the form of molecules known as ATP (for “adenosine triphosphate”). The amount of [...]

Jun
26
2011

About Autophagy

Autophagy is a process by which a cell breaks down and recycles its own components. In normally functioning animal cells, autophagy occurs at a very low level. Autophagy pathways are activated when a cell is running low on nutrients. The cell breaks down already existing proteins and other cell components into their basic building block [...]

Jun
26
2011

About Inflammation

Inflammation, what we commonly know as the swelling, redness, heat, and pain that often accompany injuries, is one of our body’s most important natural defense mechanisms against internal and external threats. The inflammatory process protects our body from damage and disease by releasing cells and mediators that combat foreign substances and help prevent infection. However, [...]

Jun
26
2011

About Glutamate Toxicity

Glutamate is a powerful excitatory neurotransmitter that is released by nerve cells in the brain. It is responsible for sending signals between nerve cells, and under normal conditions it plays an important role in learning and memory. There are two general ways, however, that glutamate can actually be damaging to nerve cells and the brain [...]