Mito Cocktail - Quinine

Here is what America's Compounding Center says about Quinine:

"Quinine, which is derived from the herb Cinchona succirubra, was historically applied topically to treat hemorroids and varicose veins. It can be prescribed by a doctor as the doctor as the brand-name medication Qualaquin to treat malaria and the tick-borne illness babesiosis. It is also taken by patients to prevent or treat nighttime leg cramps, and can be used to alleviate leg and muscle cramps in patients with mitochondrial disease. Quinine is taken at low doses of 200-300 mg once every evening for leg cramps, but is used in higher doses for the treatment of malaria and babesiosis. Quinine can cause serious side effects, including a condition called cinchonism, which may result in severe headaches, abdominal discomfort, convulsions, and vision problems. Quinine can also cause severe allergic reactions, hearing problems and ringing in the ears, fever, chills, blood abnormalities, confusion, dizziness, changes in heart rate and/or rhythm, jaundice, and kidney or liver problems."

Mito Cocktail - Magnesium Orotate

America's Compounding Center says the following about Magnesium Orotate:

"Magnesium orotate is a naturaly occurring substance involved in DNA synthesis, maintaining healthy heart function, and in producing energy for the body's cells. Magnesium orotate may be used to treat magnesium deficiency, improve exercise performance, and improve heart function in patients with cardiovascular disease. Dietary sources of magnesium orotate include root vegetables and whey. Typical dosing for magnesium orotate ranges from 500 - 2,000 mg twice daily, to be taken before meals. Magnesium orotate supplementation is generally well-tolerates, but patients may experience diarrhea."

Mito Cocktail - Uridine

America's Compounding Center says the following about Uridine:

"Uridine is naturally present in the body and is a component of RNA whichis used to produce DNA. Urisine is also involved in the breakdown of carbohydrates to produce energy. Some patients with mitochondrial diseases cannot produce uridine. A recent clinical trial has reported that uridine supplementation may improve cognition, muscle control, and kidney function in children with mitochondrial disease and was generally well-tolerated over a period of two years. The ideal dose of uridine for patients with mitochondrial disease is currently unknown. Long-term therapy with 150 mg of uridine daily has been reported as safe. Uridine supplementation may cause stomach discomfort and diarrhea."

Mito Cocktail - Ribose

America's Compounding Center says the following about Ribose: 

"Ribose is a sugar that is produced by the body from food as a source of energy. Ribose may be effective at increasing energy levels, improving ath;etic performance, improving functionality in patients with coronary artery disease, fibromyalgia, and congestive heart failure, and to prevent symptoms associated with the mitochondrial disease moadenylate deaminase (MAD) deficiency (also known as AMPD deficiency) after exercise, such as muscle pain and cramping. Ribose is usually given as a 15g oral dose four times daily, but can also be given before, during, and after exercise to prevent muscle cramping. Side effects of ribose include stomach discomfort, nausea, diarrhea, headache, and hypoglycemia."

Mito Cocktail - N-Acetyl Cysteine (NAC)

America's Compounding Center says the following about N-Acetyl Cysteine (NAC):

"N-acetyl cysteine, also known as acetylcysteine or NAC, is an antioxidant related to the amino acid cysteine. N-acetyl cysteine is commonly used to treat acetaminophen (Tylenol) overdose; however, there are many other uses for this medication. N-acetyle cysteine is also used to relieve respiratory symptoms associated with chronic bronchitis, cystic fibrosis, and reduce mucous in other lung conditions, and to prevent liver and kidney damage. It is used in patients with mitochondrial disease dues to its antioxidant effects. Dosing depends on the indication, but oral dosing for chronic conditions generally ranges from 200-600 mg two to three times daily. Side effects of n-acetyl cysteine include nausea, vomiting, diarrhea, rash, headache and drowsiness."

Mito Cocktail - Glutamine

America's Compounding Center says the following about Glutamine:

"Glutamine is a naturally occurring amino acid that is primarily produced and stored in the muscles. It helps the body produce proteins and protects the lining of the stomach. Glutamine also acts as an antioxidant and helps to protect and remove harmful waste products from the liver. Glutamine supplements are used to treat short bowel syndrome, inflammatory bowel disease, inflammation of the mouth associated with chemotherapy, to prevent nerve damage due to chemotherapy agents, to improve wound healing, and to treat glutamine deficiency in patients with mitochondrial disease and other conditions. Dietary sources of glutamine include meats, dairy products, and uncooked spinach. Glutamine is generally given orally as 5 grams (in powder, tablet or capsule form) up to six times daily, for a total daily dose of up to 30 grams per day. Patients should mix the powder with cold or room temperature liquids, as heat inactivates glutamine. Side effects associated with glutamine supplementation include rash, stomach discomfort, nausea, vomiting, swelling in the arms and legs, cough, fever, chills, and liver and kidney problems."

Mito Cocktail - NADH

America's Compounding Center says the following about NADH:

"NADH, which stands for nicotinamide adenine dinucleotide hydrate, is a naturally occurring chemical related to B vitamins that helps the body produce energy necessary for normal cell function. NADH supplements have ben used to enhance immune system function, increase energy levels, improve athletic performance, decrease blood pressure and cholesterol levels, improve nerve function in patients with Parkinson's disease, decrease the side effects of some medications used to treat human immunodeficiency virus (HIV) infection, and for conditions such as mitochondrial disease that may result in NADH deficiency. Patients typically take between 2.5 and 10 mg of NADH by mouth daily. Side effects reported with NADH supplementation are minimal and include insomnia and anxiety."

Mito Cocktail - Coenzyme Q10 (Idebenone)

America's Compounding center says the following about idebenone:

"Idebenone is a synthetic product similar to coenzyme Q10 (CoQ10) which is primarily used for its antioxidant effects. The effects of idebenone are being investigated in patients with Alzheiner's disease, optic nerve problems, a type of heart failure known as Fredreich's ataxia, and mitochonrial disease. Patients most commonly receive doses of 90-120mg of idebenone by mouth daily, but doses up to 300mg daily have been used. Side effects associated with the use of idebenone include stomach upset, nausea, vomiting, diarrhea, headache, dizziness, and confusion."

Mito Cocktail - Vitamin D3

America's Compounding Center says the following about Vitamin D3:

"Vitamon D3, also known as cholecalciferol, is a fat-soluble product which is naturally produced by the skin when a patient is exposed to sunlight. Fat-soluble vitamins are stored by the body and do not need to be replaced daily through dietary consumption.  Dietary sources of vitamin D include dairy products and fish. Vitamin D is necessary for calcium absorption and is involved in bone formation. Adequate vitamin D intake reduces the risk of bone fractures, and may reduce the risk of heart disease and certain types of cancer. Severe vitamin D deficiency may cause rickets, a condition involving softening of the bones and joint malformation due to a combination of calcium, phosphate, and vitamin D deficiency. Causes of vitamin D deficiency include malabsorption or genetic disorders, lack of exposure to sunlight, poor dietary intake, and kidney failure. Because vitamin D is a fat-soluble vitamin, doses are represented in international unitls (IU), rather than milligrams (mg). Vitamin D is commonly given orally in doses of 400-2000IU daily. Side effects are rare, and may include weakness, fatigue, headache, and loss of appetite."

Mito Cocktail - Folic Acid

America's Compounding Center says the following about Folic Acid:

"Folic Acid, also known as folate or pteroylglutamic acid, is a B vitamin involved in DNA synthesis and the production of red blood cells. In pregnanct women, folic acid is necessary for the development of the baby's spinal cord. Dietary sources of folic acid include green leafy vegetables, beef, eggs, fish, and whole grain cereals.  Folic acid deficiency may be due to alcoholism, malabsorption syndromes, genetic and mitochondrial disease, liver disease and certain medications. Symptoms of folic acid deficiency include anemia. fatigue, forgetfulness, headache, and diarrhea. The UMDF recommends a supplemental dose of 1-10 mg of folic acid daily. Folic acid is generally well-tolerated, but high doses may result in side effects such as abdominal pain and diarrhea."

Mito Cocktail - Vitamin K1

America's Compounding Center says the following about Vitamin K1:

"Vitamin K1, also known as phytonadione or phylioquinone, is a fat-soluble vitamin that plays an important role in producing specific chemicals in the liver that allow blood to clot. Fat-soluble vitamins are stored by the body and do not need to be replaced daily through dietary consumption. Dietary sources of vitamin K1 include green leafy vegetables, such as spinach, broccoli, and kale. Vitamin K1 is commonly administered in newborns to prevent bleeding, and is also used to reverse the effects of the anticoagulant medication, Warfarin. Causes of vitamin K1 deficiency include malabsorption syndromes, genetic defects, liver disease, bowel resections, and poor dietary intake. Vitamin K1 deficiency may cause unusual bleeding or easy bruising. Common doses of oral vitamin K1 supplementation range from 1-25 mg daily. Potential side effects include jaundice and allergic reactions, such as rash, hives, and airway constriction."

Mito Cocktail - NA Succinate

America's Compounding Center says the following about NA Succinate:

"Na sucinate, also known as sodium succinate or succinate, is a chemical involved in the body's production of energy required for normal cell function.  Succinate has been studied to improve mitochondrial function in patients with mitochondrial disease, and may aid in energy production and increase overall energy levels. Succinate is also an antioxidant that helps protect against damage to cells by harmful chemicals called free radicals and may even help to prevent cancer. The UMDF recommends taking 6 grams of sucinate daily."

Mito Cocktail - Biotin

Here is what America's Compounding Center says about Biotin:

"Biotin, also known as vitamin H, is a water-soluble B complex vitamin that is involved in the metabolism and conversion of food into energy. Water-soluble vitamins are not stored in the body, therefore need to be replaced regulalry in the diet. Biotin helps maintain the health of several tissues, including the eyes, skin, hair, and liver. Dietary sources of biotin include egg yolk, mushrooms, dark green vegetables, liver, and shrimp. Biotin deficiency is rare, since it is normally produced in the intestine. Biotin deficiency is usually due to chronic malabsorption, genetic disorders, or long-term therapy with total parenteral nutrition (TPN), anticonvulsants, or antibiotics. Symptoms associated with biotin deficiency include stomach upset, fatigue, muscle pain, hair loss, anemia, mood changes, and dry skin or a grayish discoloration of the skin. The UMDF recommends taking 2.5-10 mg of biotin daily. Biotin supplementaion has been associated with stomach upset, but is generally well-tolerated."

Mito Cocktail - Selenium

America's Compounding Center says the following about selenium:

"Selenium is an essential element found in trace amounts throughout the body. It is an antioxidant and may have anticancer and anti-aging effects. Selenium also helps maintain immune system and thyroid function. Dietary sources of selenium include nuts (particularly Brazil nuts), fish, shellfish, meats, and whole grain breads and cereals. Selenium deficiency is rare, but can occur in patients with malabsorption syndromes, genetic and mitochondrial disease, and in alcoholics. Complications of selenium deficiency include changes in the fingernails and nail beds, muscle weakness or pain, bleaching of the skin and hair, and heart problems. The UMDF recommends taking 25-50 micrograms (mcg) of selenium daily. Patients should not receive more than 400 mcg of selenium per day due to risk of selenium toxicity, which can result in fatigue, hair loss, and skin lesions. "

Mito Cocktail - Vitamin C

America's Compounding Center says this about Vitamin C:

"Vitamin C, also known as ascorbic acid, is a water-soluble vitamin found in fresh fruits and vegetables such as oranges, green peppers, broccoli, tomatoes, kiwi, and strawberries. Water-soluble vitamins are not stored by th ebody, therefore they need to be replaced regularly in the diet.  Vitamin C is a natural antioxidant, helps the body maintain skin and bone health, and aids in wound healing. Vitamin C has been studied for many uses, including prevention of the common cold, cancer, and heart disease, as well as treatment of high blood pressure, and acceleration of wound healing in patients with severe burns. Causes of vitamin C deficiency include poor dietary intake, cigarette smoking, malabsorption syndromes, and genetic disorders. Symptoms of Vitamin C deficiency include fatigue, petechiae, bleeding or swollen gums, dry skin, brittle nails and bone changes. In cases of severe deficiency, a patient can develop scurvy. The UMDF recommends taking 100-500 mg of vitamin C one to three times daily. Adverse effects associated with high-dose Vitamin C supplementaton include nausea, stomach upset, diarrhea, and kidney problems. "

Mito Cocktail - Cobalamin (B12)

America's Compounding Center says the following about B12:

"Cobalamin, also known as Vitamin B12 or cyanocobalamin, is a complex B vitamin involved in DNA synthesis, and in the conversion of food into energy. Dietary sources of cobalamin include meat, poultry, clams, oysters, liver, and dairy.  Since cobalamin is stored in the body, deficiency is rare and takes about three years for the body to develop. Cobalamin deficiency most commonly develops in patients with chronic malabsorption syndromes, genetic disorders, and vegetarians who do not consume any animal products. Complications of cobalamin deficiency include anemia (low red blood cell count), fatigue, muscle weakness, poor coordination, confusion, and tingling or numbness in the hands and feet. Cobalamin is available in oral, injectable, or intranasal dosage forms. A common oral dose for patients with cobalamin deficiency ranges from 100-1,000 micrograms (mcg) daily. Potential side effects of cobalamin supplementation include itching, diarrhea, headache, and anxiety."

Mito Cocktail- Pyridoxine (B6)

America's pharmacy says this about B6:

"Pyridoxine, also known as vitamin B6, is a water-soluble B vitamin involved in the conversion of carbohydrates and protein into sources of energy. Water-soluble vitamins are not stored by the body, therefore they need to be replaced regularly in the diet. Pyridoxine is also necessary to maintain healthy nerve cell function, and is involved in the production of red blood cells and hormones. Dietary sources of pyridoxine include whole grain breads and cereals, spinach, green beans, bananas, carrots, eggs, chicken, and walnuts. Pyridoxine supplementation is used to treat pyridoxine deficiency, which may be due to malabsorption or genetic disorders, alcoholism, poor dietary intake, or certain medications. Symptoms of pyridoxine deficiency include dry, peeling skin, mental confusion, nerve dysfunction, and poor coordination. Pyridoxine can also be used to treat medical conditions such as anemia and nerve disorders. Typical doses used in the treatment of pyridoxine deficiency range from 10 to 250mg daily, but doses up to 600mg daily have been used. Potential side effects of pyridoxine include and increased sensitivity to light, rash, headache, stomach upset, allergic reactions, and tingling or numbness in the hands and feet. "

Mito Cocktail - Niacin (B3)

America's Compounding Center says this about Niacine (B3):

"Niacin, also known as nicotinic acid, nicotinamide or vitamin B3, is a complex B vitamin. Niacin is used to treat patients with niacin deficiency, known as pellagra, which may be due to poor dietary intake, aging, malabsorption syndromes, or alcoholism. Classic symptoms associated with pellagra include inflammation and irritation of the skin, mental confusion, and diarrhea. Patients commonly develop a rash with darkening or thickening of the skin on the face. Niacin is found in lean meats, poultry, grains, eggs, fish, milk and legumes (such as beans, peas and peanuts). The recommended dose of niacin for mitochondrial disorders ranges from 50-100mg by mouth daily, according to the UMDF. Side effects associated with niacin supplementation include stomach discomfort, flushing of the upper body (particularly face and neck), headache, and liver problems. "

What is N-Acetylaspartic Acid (NAA)?

This is a question I've been asking over the last month. What, exactly, is this chemical, NAA, that is low in J's brain?

Simply stated,  N-Acetylaspartic Acid (NAA) is a marker of neuron integrity and viability.  A study in the American Journal of Neuroradiology said the following:  Due to its near-exclusive localization to neurons and their processes, NAA is regarded as a marker for their health and density, and its level has been reported to decline in all neurodegenerative central nervous system disorders in adults.

N-Acetylaspartic Acid even has an entire website dedicated to it.  N-Acetylaspartic Acid.com
NAA is also known as N-acetylaspartate and NAA is the second most  most abundant amino acid in the brain. NAA gives off the largest signal in MRS of the human brain. This along with the fact that it is pretty much exclusive to neurons, makes it a reliable marker.  The levels measured in the brain are decreased in many neuropathological conditions ranging from brain injury, stroke,  Alzheimer's  to Parkinson's. This also makes NAA a reliable diagnostic molecule for doctors treating patients with brain disease.

An article that I found on PubMed says: 
An article I found on PubMed says this:  "MRS studies of human brain disorders have invariably detected decreases in brain NAA concentrations when neuronal loss or dysfunction are involved with one major exception: Canavan Disease... virtually all other neurological disorders involving neuronal loss or dysfunction result in reduction of NAA levels."

A 2003 Medscape article  titled MRI Shows Brain Injury in MS That Precedes Atrophy says the following:  
"Neuronal cell death may be an important marker to measure the progression of MS, and this eventually may be a therapeutic target. "First you lose the neurons, and then the axons atrophy," Dr. Gonen told Medscape. "We don't have a treatment that interrupts this process, but physicians can use these images to encourage their patients to take medications that prevent attacks."

The marker for neuronal cell death is the amino acid derivative N-acetylaspartate (NAA). Because this protein is present almost exclusively in neuronal cells, Dr. Gonen and colleagues theorized that quantifying the level of NAA by proton MR spectroscopy would give an indication of whether the patient was experiencing a loss of these cells. Therefore, they sought to quantify the relationship among NAA levels, brain volume, and disease duration to better understand the early developments in MS."

Mitochondria and Brain Disease

Mitochondria and Brain Disease is a great article for anyone looking for the link between the two.

"It is not surprising that, in adult neurons, which depend primarily on ATP production to meet bioenergetic demands, any compromises in mitochondrial function place neurons at a high risk for both dysfunction and/or death. The association between mitochondrial abnormalities and disease has been known for approximately four decades, with the description of a patient with hypermetabolism and a skeletal muscle biopsy demonstrating large numbers of abnormal mitochondria, a disorder now termed “mitochondrial myopathy” (Cassarino & Bennett, 1999). There exists substantial evidence that mitochondrial dysfunction and oxidative damage may play a key role in the pathogenesis of neurodegenerative disease. Evidence implicating both mitochondrial dysfunction and oxidative damage in the pathogenesis of Alzheimer’s disease (AD), and Huntington’s disease (HD), as well as ischemia and other neurological disorders, continues to accumulate"