Mighty Mitochondria – The Body’s Master Regulators

Tiny Mitochondria Are Mighty

Our mighty mitochondria perform some of the most amazing aspects of health because of their ability to convert food into energy. Mitochondria are tiny, microscopic “organelles” (essentially ancient bacterium passed down from mother to child that have their own DNA, different from ours) that populate nearly every cell of our body, red blood cells being a notable exception. Their functions are critical for achieving and maintaining a well-regulated body equipped with enough energy to not only survive but to really thrive.

Mitochondria are primarily responsible for the conversion of the food we eat – carbohydrates, fats and proteins – into our body’s main energy currency, ATP (adenosine triphosphate). ATP is the fuel that makes our bodies function. The number of mitochondria that inhabit any given cell in our body depends on that cell’s energy needs. For example, there may be 1,000-2,000 mitochondria in each individual liver cell, or 5,000-8,000 in just one heart cell, or 2 million in brain cells (neurons).

The ATP molecule is created through a process called cellular respiration that allows it to carry, store and release energy to cells in need. When mitochondria are compromised, this process is distorted at a cellular level: the very tissues that make up critical organs are less capable of producing and utilizing ATP, and they lose functionality.

Life-Generating Mitochondria

Because of their ability to generate energy, mitochondria play a significant role in the lives of cells throughout the body systems:

• Mitochondria help the neurovascular system by:
  • Supporting cognitive and neurological development
  • Supporting calcium signaling for nerves and muscles
  • Regulating the production of neurotransmitters such as catecholamines used in the “fight or flight” response
  • Regulating excitotoxicity by balancing GABA and glutamate
• Mitochondria support tissue growth and repair:
  • Helping to regulate cell growth and development
  • Invoking apoptosis (cell death) to get rid of unwanted (senescent) cells
• Mitochondria enable the immune system by:
  • Responding to adversity (e.g., pathogenic viruses, bacteria etc.)
• Mitochondria allow the endocrine system by:
  • Maintaining appropriate body temperature and homeostasis
  • Regulating stress hormones such as cortisol and
  • Regulating reproductive hormones such as estrogen, progesterone and testosterone
• Mitochondria complement detoxification/waste removal systems by:
  • Supporting the high energy demands of the emunctories (routes of elimination) such as the gastrointestinal tract, liver, kidneys, lymphatics, skin, lungs and uterus

Given this small sample of their roles, it is no surprise that mitochondria are often called the “powerhouse of cells” or “the master regulators”.

Mitochondria, Oxidative Stress, Inflammation, and Free Radicals

As with manmade power plants, mitochondria also produce potentially harmful “waste” – byproducts called free radicals that are extremely reactive. These highly volatile molecules need to be removed, recycled or mitigated in some way by the body. When the total load of stressors on a body is too great or the demands on the mitochondria outweigh their capacity to deliver, these byproducts may be left “unchecked” and accumulate beyond the body’s capacity to manage them effectively. A subset of free radicals, called reactive oxygen species can cause significant damage to DNA, protein, and other cellular components.

When the body encounters too many stressors in any form, such as a high total load, the number of free radicals rises exponentially, leading to more oxidative stress which triggers an inflammatory response, which leads to more oxidative stress, which can compromise the body’s biological systems, cells, tissues and organs and harm the mitochondria itself. This vicious cycle further damages cells, proteins and DNA and contributes to many chronic conditions and diseases.

If antioxidants – molecules in the body that help mitigate oxidative stress which can be found in many fruits, vegetables, and supplements – are not sufficient to meet the body’s demands, oxidative stress overtakes the body’s defenses and leads to oxidative damage of whole systems. For example, even a very small change in mitochondrial function can have a profound impact on brain, muscle and heart functioning.

Signs and Symptoms of Mitochondrial Dysfunction

Medical practitioners may suspect mitochondrial dysfunction when there are a collection of signs and symptoms from the following list of examples (roughly grouped into three categories):

Neuromuscular and/or Developmental Issues

• Low muscle tone (hypotonia)
• Difficulty swallowing or feeding
• Muscle wasting
• Headaches
• Fainting
• Seizures
• Alzheimer’s disease
• Parkinson’s disease
• Fibromyalgia

Constitutional Problems and/or Chronic Health Conditions

• Cardiovascular disease
• Insulin resistance
• Metabolic syndrome
• Diabetes
• Obesity
• Nutrient deficiencies
• Chronic and/or recurring infections

Other Signs and Symptoms Related to Specific Body Systems

• Psychiatric disorders
• Depression
• Bipolar disorder
• Schizophrenia
• Poor diet
• Lack of activity

A Deeper Look at Hypotonia and Muscle Tone

Enough can’t be said about how important mitochondria are to muscle function. Mitochondria are essential for muscle tone, growth and development. When mitochondria are unable to produce enough energy inside muscle cells, muscle weakness and hypotonia may present. Children with low muscle tone are often described as having hypotonia. When babies experience muscle weakness, it may affect their ability to feed. Babies with feeding difficulties may be experiencing mitochondrial dysfunction in addition to other challenges such as tongue tie. Hypotonia or mitochondrial challenges are common in conditions such as Down Syndrome, Prader-Willi, muscular dystrophy and cerebral palsy.

Muscles require a huge amount of constant energy to maintain strength and function; otherwise, they can become floppy and weak. Children with mitochondrial disorders exhibit low muscle tone in many forms such as an inability to sit, stand or walk, or in their ability to hold pencils, write by hand, draw pictures, tie shoelaces or button shirts.

It’s important to understand the vital role of mitochondria to address a child’s health. Improving a child’s muscle tone (with multiple supports, including nutrition and targeted supplements, discussed below) can help with a variety of conditions and may help children meet their developmental milestones.

Mitochondrial Dysfunction and Autism

Mitochondrial dysfunction clearly impacts many of the body’s energy systems in children with autism. Highly impacted areas tend to be neurologic (not just the brain, but also systems such as the autonomic, enteric, and central nervous systems), cardiovascular, and musculoskeletal. Lack of ATP can contribute to many of these issues seen in autism:

• Heart/liver/kidney conditions
• Respiratory conditions
• Gastrointestinal conditions
• Auditory processing
• Motor planning
• Cognitive skills
• Social skills
• Seizures

When there is not enough ATP, children with autism may present with normal muscle tone in the lower extremities of their bodies but lack sufficient muscle tone in the upper parts of their bodies. If this is the case, they may lack fine-motor skills needed for speech.

More About Speech

Parents are usually extremely concerned when their children are non-speaking or have limited language. Lack of speech and language are very emotional topics, and as we know, the inability to communicate can have a debilitating effect on a child’s development and quality of life. There may be different biochemical reasons for speech delays in a child; it is valuable to investigate nutritional and other root causes, too.

An important piece of speech and language that is often overlooked is the role of the mitochondria and its respiratory complexes – the steps it takes to make ATP. You might already have guessed what speech and language have to do with mitochondria: muscle tone. A common issue for children with autism is that their bodies lack the energy to create enough muscle tone for them to develop and support a speaking voice.

As with their muscles, children’s brains require an enormous amount of energy, especially at a young age when the brain is still developing. Inadequate cellular energy affects how the brain cells connect and “talk” to one another and our ability to talk– period – communication is impacted on many levels.

More About Speech and Respiratory Complexes

Respiratory complexes are a crucial part of mitochondria. They make up an electron transport chain (or sequence of enzymatic steps needed to make ATP) that consists of four major protein complexes I, II, III and IV.

Children with neurodevelopmental disorders such as autism often have defects in respiratory complexes II and III, impacting the production of ATP. Low energy, in turn, can affect how brain cells connect and communicate impeding a child’s developmental milestones. For example, an important area of the brain needed for speech is called Broca’s area. It requires huge amounts of energy. Children who have impairment with complexes II and III manifest problems in delayed speech, poor motor coordination and reduced cognitive processing.

Testing and Nutritional Supplementation

Two important tests to determine underlying mitochondria issues or needs are the MitoSwab by Religen Diagnostics and the Organic Acid Test (OAT). Both tests can help medical providers pinpoint the source of signs and symptoms associated with mitochondrial dysfunction.

Another valuable test checks for antibodies to the brain’s folate receptors in a blood sample. It is called the FRAT (Folate Receptor Antibody). If these antibodies are present, they can interfere with speech production and could also suggest the need to avoid dairy.

The following nutrients are often used to support healthy mitochondria; a root-cause-oriented medical provider will likely develop a custom mitochondrial cocktail from these nutrients based on a child’s lab test results and bio-individual needs:

• Superoxide Dismutase (SOD)
• D-ribose
• NADH (Co-Enzyme 1)
• PQQ Pyrroloquinoline Quinone
• Alpha Lipoic Acid (ALA)
• N-Acetyl Cysteine (NAC)
• Nicotinamide adenine dinucleotide (NAD+)
• Polyphenols

Supporting Mitochondria through Daily Activities

Before jumping into the many things that can harm mitochondria, here is a review of some approaches that have been shown to support them. The following are examples of ways to help facilitate optimum functioning:

• Circadian rhythm optimization
• Daily sunlight exposure
• Dietary changes
• Relaxation techniques

A Special Look at the Need for L-Carnitine

L-carnitine is an extremely important element of protein from our diets needed by mitochondria to create energy. It is an amino acid that is necessary to transport long-chain fatty acids across the inner membrane into mitochondria, where they are broken down and the fatty acids are turned into ATP energy. Fatty acids are unable to get into the mitochondria without L-carnitine.

Most people acquire L-carnitine through diet such as red meat (the best source), poultry, fish and dairy products. A carnitine deficiency in infancy or childhood, however, could show symptoms such as:

• Severe brain dysfunction or encephalopathy
• A weakened and enlarged heart (cardiomyopathy)
• Vomiting
• Muscle weakness
• Low blood sugar

L-carnitine also prevents toxic build-up in the mitochondria and in so doing supports overall cellular health. It is also involved in more than 100 enzymatic reactions including those used in these processes:

• The citric acid cycle
• Maintaining a Coenzyme A balance to create ATP
• Reducing oxidative stress
• Protecting the mitochondrial membranes from damage
• Breaking down fatty acids into ATP

We can see why there cannot be a discussion about mitochondria without stressing the importance and necessity of L-carnitine.

What Is Toxic to Mitochondria?

Unfortunately, today we are surrounded by stressors and toxins that damage mitochondria. Many diet, lifestyle and environmental factors, play a part in contributing to our mitochondria’s total load, including:

• Artificial environments full of WiFi radiation and other EMFs
• Lack of nutritious foods or too many processed foods and food dyes
• Poor quality fats (including oxidized oils) or low intake of high-quality fats
• GMO foods, especially those treated with pesticides/glyphosate
• Pathogens or an imbalanced microbiome (known as gut dysbiosis)
• Low sun exposure, poor air quality, or exposure to fluorescent light
• Artificial blue light from screens or lack of natural infrared light
• Chronic circadian misalignment – being out of rhythm with nature

What Is Happening at a Cellular Level?

As a physician-scientist, Robert Naviaux’s groundbreaking research proposed that mitochondria respond to these toxic stressors as a threat. He called this the “Cell Danger Response” (CDR). In 2013, Dr. Naviaux published a paper about CDR as a theory about autism showing that he was able to reverse behaviors and biochemical abnormalities of autism in mouse models. He later published studies about how a 100-year-old drug called suramin to lower that danger signal. Dr. Naviaux feels much work needs to be done to perfect his solution, but he remains certain on this thought:

According to Doug C. Wallace, a geneticist and evolutionary biologist, today’s environmental toxicity accelerates oxidative stress to create more dysfunctional mitochondria.

Dr. Wallace also maintains that “Disease is a bioenergetic collapse, not a genetic destiny.” In other words, “disease” is the energetic downfall which causes the collapse of mitochondria rather than genetic etiology. The focus should be on the restoration of mitochondria, not on “bad genes”.

Both conventional and integrative practitioners, from psychiatrists to pediatricians to neurologists, are beginning to focus on mitochondria as a key issue for better understanding where/how health problems arise and how to address them. This is very significant progress for all health arenas and stages of life. These practitioners are helping their patients by getting to their root causes, not just addressing patient symptoms.

Conclusion

If you are raising children, it is valuable to consider the health of their mitochondria and to make sure they are getting enough protein, healthy fats and other nutrients described above to support their mitochondria. It is also important to lower their total load of stressors. If you also “lack energy” or are one of those people categorized as having a “slow metabolism,” you can potentially increase your metabolism by supporting your own mitochondria. If you do nothing else new for you or your child’s body, consider supporting the mitochondria! These organelles are a gateway to life and key to all bodily functions that require energy. They truly do allow us to survive and thrive. This is why they deserve the title “mighty mitochondria”.

About Heather Tallman Ruhm MD

About Teresa Badillo

She won a court case in 1995 against the Rockland County School District in New York to enable ARC Prime Time for Kids to be the first school using Applied Behavioral Analysis teaching method for autism that was paid for by the Rockland County School District. The following year she was instrumental in getting the New York Minister of Education to approve an extension of the ARC license from 5 to 21 years.

She has worked over the years in a number of alternative medical practices with doctors and practitioners organizing various biomedical intervention options for children with autism. Since the mid 1990s, Teresa has served on several boards:

• Foundation for Children with Developmental Disabilities
• The Autoimmunity Project
• Developmental Delayed Resources
• Epidemic Answers

She continues to consult and advise parents on all different areas of autism especially nutritional protocols. Since 2006 she has worked with NutraOrgana, LLC and BioCellular Analysis Testing. She currently researches, writes the newsletter and blogs Teresa’s Corner for The Autism Exchange (AEX). She also writes blog posts and pages for Documenting Hope.

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