When the cell structure becomes diseased: mitochondriopathy

Many body cells have a cell structure called a mitochondrion. They are used by the cell to generate energy. In mitochondriopathy, the energy metabolism is disturbed, which means that little energy is produced. The reason for this is a mutation in the genetic material. Mitochondriopathy can vary in severity and have many different symptoms. Taking Neotes α can help to support the mitochondria in their natural function.

What are mitochondria?

The mitochondrion is a cell organelle with its own genetic material. It is found in all body cells that have a cell nucleus (also known as eukaryotic cells). Mitochondria are mainly found in cells with high energy consumption, including:

• Muscle cells

• Sensory cells

• Ova

• Nerve cells

The main task of mitochondria is in metabolic processes such as the respiratory chain. In this process, energy for the cell is obtained from sugar or fat. Proteins are also involved in this respiratory chain. If there is a so-called mitochondriopathy due to genetic mutations, energy production is disrupted. This in turn leads to the disruption of organs that require a lot of energy, such as the nervous system or the eyes.

How does mitochondriopathy manifest itself?

The first symptoms of the disease often appear in childhood and adolescence. However, the disease can occur at any age. The disease is one of the rare diseases and affects around one in 5000 people.

If the mitochondrion’s energy production is disrupted, this first leads to functional disorders and ultimately to cell death. Mitochondriopathies can affect any organ and cause a variety of symptoms. In most cases, the symptoms worsen as the disease progresses. These symptoms include, for example

• Muscle cramps or weakness

• Visual disturbances

• epileptic seizures

• Gastrointestinal disorders

• Diseases of the heart or liver

• Diabetes

• Susceptibility to infection

• Short stature

• Stroke-like episodes

In mitochondriopathies, there is a genetic change in the DNA of the mitochondria, or the cell nucleus. This mutation is either inherited or spontaneously acquired.

Over 250 genes are now known to be the cause of mitochondriopathies. However, it is assumed that a larger number are still unknown. Even though mitochondria have been known since 1886, mitochondrial diseases have only been diagnosed since the 1960s.

Research into mitochondriopathies is extremely important

The tiny mitochondria appear to play a major role in human health. Every disease associated with an energy problem can ultimately be traced back to them. A lot of known chronic degenerative diseases are linked to mitochondrial dysfunction:

• Alzheimer’s disease

• Parkinson’s disease

• Huntington’s disease

• ALS

• Cardiovascular diseases

There is no evidence (to date) that mitochondriopathies cause these diseases, but it is definite that they are involved. It is now also assumed that autoimmune diseases such as multiple sclerosis, rheumatoid arthritis, lupus or Sjögren’s syndrome are based on mitochondrial disorders. Mitochondriopathies are also associated with a wide range of tumors.

What to do with mitochondriopathy?

If there is a well-founded suspicion of mitochondriopathy based on the symptoms, further examinations are carried out in specialized centers. Various blood tests are carried out. In addition, the organs that have probably been affected are checked. Hormonal disorders often occur in patients with mitochondriopathies. It is therefore important to regularly determine the concentration of hormones.

A muscle biopsy provides definitive information about the disease. A tissue sample is taken under local anaesthetic and analyzed under a microscope.
Affected families are advised to visit a human genetic counselling center. A family tree is drawn up there, which can be used to identify other affected individuals. The risk of offspring can also be calculated there.

In principle, mitochondriopathy cannot be cured. Treatment consists of alleviating the symptoms and avoiding complications. Depending on the symptoms, these are treated specifically (diabetes, epilepsy, etc.).

One promising option is to support the mitochondria in their work. This can be done with the help of the vital substance Neotes α. The α-ketoglutarate it contains is involved in many cellular processes and metabolic processes. The special formulation of Neotes α is also used in longevity research.

In addition, endurance sports have proven effective for mitochondriopathies. However, those affected should keep a close eye on their exercise limits. If the disease is diagnosed very early, it usually progresses more quickly. Nevertheless, optimal therapy, supplemented with vital substances, can improve the quality and expectation of life.