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MitoQ Trial For Chronic Fatigue Syndrome, Fibromyalgia And Mitchondrial Disorders Begins
Wednesday 18 November 2015
MitoQ Trial For Chronic Fatigue Syndrome, Fibromyalgia and Mitchondrial Disorders Begins
Could mitochondrial problems be key to explaining CFS/FM symptoms?
A recent blog entry by Cort Johnson, founder of the Chronic Fatigue Syndrome/Fibromyalgia Syndrome (CFS/FM) activism site Health Rising, a non-profit corporation since 2010, draws readers’ attention to theories that mitochondrial problems may underlie some manifestations of these disorders.
Mr. Johnson, a CFS/FM sufferer himself, observes that chronic fatigue syndrome (AKA myalgic encephalomyelitis or ME) and fibromyalgia can cause so many symptoms that they can elicit a variety of diagnoses — a problem — but he suggests that the sheer number of symptoms found in these disorders may actually be a helpful clue. Not many diseases can produce such a ‘rich broth’ of symptoms.
He notes that not many disease can produce the wide spectrum of symptoms that CFS/FM typically do, tending to have systemic (body-wide) impacts or affecting the brain, with associated diagnoses such as as autoimmune disease, post-viral syndrome, neurodegenerative disorders, so-called functional syndromes (FM, ME/CFS, etc.) and/or some mood disorders.
But “what about mitochondrial disorders?” Johnson asks rhetorically. “Hundreds of mitochondrial diseases exist, but many do have systemic effects. In fact, the United Mitochondrial Disease Foundation (UMDF) recommends that doctors think mitochondrial disease when three or more organ systems are affected. The Foundation also lists CFS-like illnesses as possible indicators of a mitochondrial disease.”
The term Mitochondrial disease refers to a broad range of disorders involving mitochondrial dysfunction, with many more believed to have yet to be discovered. Because mitochondria perform so many different functions in different tissues, there are literally hundreds of different mitochondrial diseases that are variously estimated to affect at least one in 6000 persons, and possibly as many as one in 4000, with approximately 20,000 people in the United States believed to have a form of mitochondrial myopathy, and approximately 1,000 to 4,000 American children born with the disorder, for which there is currently no cure, each year.
Mitochondria (singular mitochondrion) are described in literature as tiny subunits present inside every cell of the human body except red blood cells, from the skin’s surface to deep internal organs. Mitochondria’s main role transforming food and oxygen that enter the cells, and they are responsible for creating more than 90 percent of the energy needed by the body to sustain life and support. The term mitochondrion derives from the Greek mitos (“thread”), and chondrion (“granule” or “grain-like”). More technically, mitochondria are double membrane-bound organelles — specialized subunits within cells with a specific function that are found in most eukaryotic cells, that is: cells that have a nuclear envelope, cytoplasmic organelles, and a cytoskeleton.
Symptoms cited as associated with mitochondrial disease include muscle weakness and pain, gastrointestinal disorders, fatigue, lack of endurance, and exercise intolerance
Cort Johnson notes that post exertional malaise (PEM) or relapse is a major problem in ME/CFS and FM and also occurs in people with mitochondrial diseases, although whether they experience the same kind of PEM that people with ME/CFS or FM experience is unclear. However, he notes that “the “global fatigue” people with mitochondrial disorders can experience after exertion, which causes slowed thinking, confusion (and in some “an unmasking of behaviors normally under control”) sounds very much like ME/CFS and FM.”
Moreover, Johnson observes that people with mitochondrial diseases are, by definition, low in energy – at least in some parts of their bodies the muscle symptoms after exercise (pain, cramping and/or muscle spasms with or without tenderness and/or feelings of heaviness (‘cement legs’) particularly in the muscles used) bear some similarities to mitochondrial disease symptom profiles, and that signs of significant autonomic dysfunction, such as rapid or slowed heartbeats, problems standing, heat or cold intolerance, unusual sweating, spontaneous pallor or flushing or mottling and gut and bladder problems appear to be often found in both mitochondrial diseases and ME/CFS/FM, as do sleep problems including sleep apnea, unrefreshing sleep and daytime fatigue, which are common in mitochondrial disorders and ME/CFS and FM, as is, unsurprisingly, emotional distress.
The National Institutes of Health (NIH) says that while there is no specific treatment for any of the mitochondrial myopathies, physical therapy may extend the range of movement of muscles and improve dexterity, and vitamin therapies such as riboflavin, coenzyme Q (CoQ), and carnitine (a specialized amino acid) may provide subjective improvement in fatigue and energy levels in some patients.
Cort Johnson points to CoQ10 playing a critical role in generation of aerobic energy (ATP) – the source of about 95 percent of the energy in our bodies, citing several studies suggesting that low CoQ10 levels could be contributing factors in symptoms found in fibromyalgia as well, including a Spanish trial that resulted in significant reductions in pain, fatigue and morning tiredness as well as reduced inflammation and increased mitochondrial activity. He says similar findings were reported in a Japanese trial of adolescents with FM and CoQ10 reduced pain and headache in FM patients in a third. Finally, he notes that a small study found evidence of both mitochondrial dysfunction and CoQ10 depletion in the tissues of FM patients.
Also pertaining to coenzyme Q for CFS/FM, the currently active Fibromyalgia & Chronic Fatigue Syndrome MitoQ Study is a double-blind, placebo-controlled trial examining the influence of a the mitochondria targeted proprietary supplement product MitoQ on CFS and FM symptoms. Participants in the study created by a researcher and ME/CFS patient, Joshua Grant, whose MENDUS site allows health communities to create their own online clinical trials, are being asked to record symptoms and take three cognitive tests 5x over a period of several weeks, the study protocol being based around recently CoEnzyme Q10 studies. The study is active, and participants can sign up and begin at any time, Free spaces in the MitoQ trial have now been filled, as have spaces in which MitoQ’s manufacturer had been was offering 50 percent off for additional people who wished to participate. However, it’s still possible to join the trial using self-purchased MitoQ obtained at a reduced price on the MENDUS site.
MitoQ is claimed by its manufacturer to be a mitochondrial targeted antioxidant that that acts directly in mitochondria as an antioxidant against free radicals by delivering antioxidant CoQ directly to the mitochondria, supporting their natural function and restoring their health, thus helping them to get on with their job by eliminating free radicals, preventing oxidation, and keeping the skin and organs healthy. MitoQ is claimed to be “847 times more effective” than any other CoQ formulation on the market.
The company explains that MitoQ is produced by binding a form of Co Q10 called ubiquinone to a fat soluble, positively-charged molecule, and that this molecule is able to flow directly into the mitochondria and through the normally impermeable inner membrane to end up deep inside the mitochondria. They note that inside of the mitochondria and inner membrane is the major site for biochemical reactions, including cellular respiration, so this puts MitoQ exactly where it is needed most, at concentrations several hundred-fold higher than if it just stayed in the blood. They claim that a reaction inside the inner membrane converts the ubiquinone in MitoQ into ubiquinol, the antioxidant and active form of CoQ10, allowing it to neutralise free radicals that accumulate within the mitochondria.
Furthermore, they say MitoQ is one of the most-studied mitochondrial-targeted antioxidants, with research having shown that after oral administration, MitoQ rapidly accumulates in mitochondria-rich tissue such as the heart, brain, skeletal muscle, liver, and kidney and supports a range of conditions associated with oxidative stress. Cort Johnson contends that calling MitoQ a mitochondrial supplement is not really doing it justice, given that it’s been featured in 180 research papers, ergo: it’s a serious mitochondrial supplement. Scholarly references and research data cited include:
Smith R, Hartley R, Cocheme H, Murphy M. Mitochondrial pharmacology. Trends in Pharmacological Sciences 2012;33(6):341-352
Smith R, Murphy M. Animal and human studies with the mitochondria-targeted antioxidant MitoQ. Annals of the New York Academy of Sciences 2010;1201:96-103
For more information, visit:
For greater detail on the study, see:
The Pittsburgh, Pennsylvania, based United Mitochondrial Disease Foundation says that while mitochondrial diseases are most frequently diagnosed in children, typically occurring before the age of 20, adult onset is becoming more and more common, and these disorders even more complex in adults because detectable changes in mtDNA occur as we age and, conversely, the aging process itself may result from deteriorating mitochondrial function, with a broad spectrum of metabolic, inherited and acquired disorders in adults in which abnormal mitochondrial function having been postulated or demonstrated.
The UMDF contends that while conventional teaching in biology and medicine is that mitochondria function only as “energy factories” for the cell, this categorization is a mistaken over-simplification that has slowed progress toward understanding the biology underlying mitochondrial disease. The UMDF notes that it takes about 3000 genes to make a mitochondrion, and mitochondrial DNA encodes just 37 of these genes, with the remaining preponderance of genes encoded in the cell nucleus and the resultant proteins are transported to the mitochondria. Ergo: only about three percent of the genes necessary to make a mitochondrion (ie: 100 of 3000) are allocated for making ATP, while more than 95 percent (ie: 2900 of 3000) are involved with other functions tied to the specialized duties of the differentiated cell in which the mitochondrion resides, and these duties change as we develop from embryo to adult as our tissues grow, mature, and adapt to the postnatal environment. It is these other, non-ATP-related functions that are intimately involved with most of the major metabolic pathways used by a cell to build, break down, and recycle its molecular building blocks, and the UMDF notes that cells themselves can’t even make the RNA and DNA they need to grow and function without the function of mitochondria. The building blocks of RNA and DNA are purines and pyrimidines, and it is the mitochondria that contain the rate-limiting enzymes for pyrimidine biosynthesis (dihydroorotate dehydrogenase) and heme synthesis (d-amino levulinic acid synthetase) required to make hemoglobin.
The UMDF maintains that mitochondrial diseases are the result of either inherited or spontaneous mutations in mtDNA or nDNA which lead to altered functions of the proteins or RNA molecules that normally reside in mitochondria, but that problems with mitochondrial function, however, may only affect certain tissues as a result of factors occurring during development and growth that we do not yet understand, and that even when tissue-specific isoforms of mitochondrial proteins are considered, it is difficult to explain the variable patterns of affected organ systems in the mitochondrial disease syndromes seen clinically.
They observe that because mitochondria perform so many different functions in different tissues, there are literally hundreds of different mitochondrial diseases. Each disorder produces a spectrum of abnormalities that can be confusing to both patients and physicians in early stages of diagnosis. Because of the complex interplay between the hundreds of genes and cells that must cooperate to keep our metabolic machinery running smoothly, it is a hallmark of mitochondrial diseases that identical mtDNA mutations may not produce identical diseases. Genocopies are diseases that are caused by the same mutation but which may not look the same clinically.
Researchers at the National Institutes of Health report the first clear evidence that muscle cells distribute energy primarily by the rapid conduction of electrical charges through a vast, interconnected network of mitochondria in a way resembling the wire grid that distributes power throughout a city. The study, published the journal Nature, entitled: “Mitochondrial reticulum for cellular energy distribution in muscle“ (Nature 523, 617620 (30 July 2015) doi:10.1038/nature14614) provides an unprecedented, detailed window showing how the system that rapidly distributes energy throughout the cell to power muscle contraction functions. These observations solve the problem of how muscles rapidly distribute energy in the cells for movement.
The above originally appeared here.
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