Arches. Photo by Daniel Chia
HOPES: Huntington's Outreach Project for Education, at Stanford
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
29
2010

TUDCA: Tauroursodeoxycholic Acid

What in the world do black bears have to do with treating Huntington’s disease? Believe it or not, a compound found in large quantities in the bile (a digestive fluid) of black bears may help prevent the death of brain cells in people with HD. (TUDCA) Tauroursodeoxycholic acid is also found in small quantities in [...]

Jun
29
2010

Carnitine

Drug Summary: Carnitine acts to facilitate the entry of fatty acids into the mitochondria. Once these fatty acids are in the mitochondria, they can be used to produce energy. Because inefficient energy production is believed to contribute to the progression of HD, carnitine therapy could result in increased energy production, and could possibly delay HD [...]

Jun
29
2010

Riboflavin

Drug Summary: Riboflavin acts as an integral component of two coenzymes: FAD (flavin adenine dinucleotide) and FMN (flavin mononucleotide). These flavin coenzymes are critical for the metabolism of carbohydrates, fats, and proteins into energy. Because riboflavin is an important component of these flavin coenzymes, riboflavin supplementation is believed to increase the efficiency of energy metabolism in cells.

Jun
29
2010

Dichloroacetate

Drug Summary: Dichloroacetate stimulates an enzyme called PDC that is essential for the production of energy in cells. Because inefficient energy production is believed to contribute to the progression of HD, dichloroacetate therapy could result in increased energy production, and could possibly help delay HD progression. The altered huntingtin protein seen in the nerve cells [...]

Jun
29
2010

Nicotinamide

Drug Summary: Nicotinamide (also referred to as Vitamin B3) is believed to cause improvements in energy production due to its role as a precursor of NAD (nicotinamide adenosine dinucleotide), an important molecule involved in energy metabolism. Increasing nicotinamide concentrations increase the available NAD molecules that can take part in energy metabolism, thus increasing the amount of energy available in the cell.

Jun
29
2010

Creatine

Drug Summary: Creatine (Cr) is a molecule produced naturally in the human body at a rate of about one gram per day in the liver, pancreas, and kidneys. People also consume about one gram of creatine per day in their diets, mostly from meat. Creatine is distributed throughout the body, but about 95% is found [...]