COENZYME Q10 (Ubiquinone)

Coenzyme Q10 (CoQ10) is a relatively new molecule in science. It was discovered in 1957 at the Enzyme Research Center at the University of Wisconsin It was isolated from beef heart mitochondria by Fredrick Crane It was soon found to be in all cell membranes and all cell organelle membranes, in all species in the animal and plant kingdoms, In man, it is produced in all cells in the first two decades of life and decreases gradually thereafter. It is in most of the food that we eat especially meat protein and dark green leafy vegetables. It is found in beans, peas, and large nuts, but not in small grains. Formula and milk have no CoQ10, but do have the nutritional substrates required for CoQ10 synthesis. Processing and cooking foods reduces the CoQ10 content.

C0Q10 has five known functions in the body:
1. Responsible for 95% of the energy produced in the body.
2. Potent intracellular antioxidant.
3. Prevents atherosclerosis
4. Prevents abnormal protein synthesis and thus age related degenerative diseases.
5. Stabilizes all cell membranes.

Its role in energy synthesis was part of a Nobel Prize awarded in 1978 to Sir Peter Mitchell.

CoQ10 is a large molecule, soluble only in lipids, thus poorly absorbed In the body. Dry powder CoQ10 is poorly absorbed, not only because of its large size but also because it will not dissolve in lipids at body temperature. Both the dry powder CoQ10 and the crystalline CoQ10 in an oil base are poorly absorbed. The new Iiquid CoQ10 softgel form has a 900% greater absorption than these do. It is the form currently being used in children with PWS.
 

MITOCHONDRIA-ENERGY PRODUCING ORGANELLES
FOUND IN ALL LIVING CELLS

Energy is strictly a mitochondrial function. Mitochondria are found in all living cells. They are small bean-shaped structures with outer and inner membranes. Five percent of the total body energy is produced In the breakdown of food substances to NADH, which occurs in the outer membrane of the mitochondria. The inner membrane is the location for Complexes I, II, and III in the synthesis of energy. The remaining ninety-five percent of energy is produced here in the presence of oxygen. This occurs when NADH (from the outer membrane) is converted to energy (ATP) through electron transfer. CoQ10 and Cytochrome C are the mediators for electron transfer through Complex II and III. Of these three intermediates (NADH, CoQ10, and Cytochrome C), CoQ10 is the only one found to be deficient in patients with low energy syndromes, including PWS.

Multiple genetic and non-genetic mitochondrial disorders have been described. In many of these clinical conditions, low CoQ10 content has been found in the body. Deficiencies of CoQ10 result in reduced energy synthesis. Under these conditions, the mitochondria show morphological changes in size, shape and location. They dry up to raisin-like organelles and are found clumped together rather than being distributed evenly throughout the cell. This results in clinical conditions that effect infants, children, and adults. Included in these are PWS, muscular dystrophy, multiple sclerosis, Huntington's Chorea, chronic fatigue syndrome, Parkinson's disease, hyperthyroid disease, heart failure, some forms of cancer, and effects of excessive drug therapy with certain pharmaceuticals.

DNA is found in all mitochondria. It is responsible for reproduction of mitochondria, which occurs under conditions of increased cell activity such as exercise and training. Trained individuals have higher numbers and greater distributions of mitochondria in skeletal muscle compared to non-trained individuals. Under conditions of muscle dysfunction (such as hypotonia and atrophy) the mitochondria have been shown to be abnormal and non-functional. In the case of CoQ10 deficiencies, this is known to occur in adults when blood levels are reduced to 0.55ug/ml or less. CoQ10 supplementation in individuals with known deficiencies significantly increases mitochondrial energy synthesis and results in an improved clinical condition and quality of life.
 

LOW ENERGY SYNDROME

Low energy syndromes have been linked to poor nutrition, digestive disorders, endocrine disorders, Vitamin B deficiencies, mitochondrial dysfunction, age and CoQ10 deficiency.

CoQ10 deficiency occurs with age. This is probably due to two things, either a dietary deficiency in the substrates required to make CoQ10 or an overall dysfunction of the biochemical mechanisms responsible for CoQ10 synthesis. It is uncommon for children and young adults to be deficient in CoQ10. In infants with PWS, there appears to be an inability of the body to produce and/or utilize CoQ10. Without adequate CoQ10, energy synthesis in the inner membrane of the mitochondria is limited. Thus, in children with PWS who have CoQ10 deficiency and normal mitochondria, CoQ10 supplementation should enhance energy synthesis and therefore metabolic activity in all body systems. If the mitochondrial mechanism is undeveloped or nonfunctional, then CoQ10 supplementation may have limited effects unless the mitochondrial function can be revived.

We have one example of the benefits of CoQ10 supplementation in a child with PWS who has both a chromosome 15 abnormality and a Complex II and II involvement. This child, for the first six months of life, did not cry nor move and was fed via a NG tube. Within three days of starting CoQ10 supplementation, the child began to develop sucking reflexes and move arms, hands, legs, and feet. With continued CoQ10 supplementation, crawling, standing and walking occurred between eight and twelve months. Today after three years of CoQ10 supplementation, in the absence of any nutritional, hormonal or drug intervention this child has normal physical and mental development. Speech patterns, physical skills, and cognitive functions are in the normal range for the age level.
 

THE MITOCHONDRIA-LOW ENERGY-CoQ10 STORY