What are Mitochondria?
The mitochondria are the 'powerhouses' of the cell
and are contained in all human cells. They perform the vital task of generating adenosine triphosphate (ATP), the molecule
the cell uses for the bulk of its energy needs. Mitochondria are responsible for creating 90% of the energy needed by the
body to sustain life and support growth.
What do Mitochondrial Do?
In a process called oxidative phosphorylation,
ATP is made from ADP and inorganic phosphate. In other words, mitochondria burn food in the presence of oxygen to produce
the energy our bodies need to sustain life and support growth.
When there is a problem in this process, energy
deficiencies result and premature cell death and organ shutdown can occur.
About Mitochondrial Disease
There are many different mitochondrial diseases into
which research is relatively new The first disease was named in 1962. Although the underlying characteristic
of these diseases is a lack of adequate energy to meet cellular needs, the effects of mitochondrial diseases on
organ systems vary considerably from one disease to the other and from case to case. The age of onset, as well
as the rate of disease progression, even within families whose members have identical genetic mutations can also vary.
Mito diseases also vary considerably in their manifestations,
leading to uncertainty about diagnosis and classification. There are no absolute diagnostic criteria for mitochondrial
cytopathies, and most screening tests are neither specific nor sensitive, which can lead to false-positive and false-negative
diagnoses.
There are currently no cures for mitochondrial
diseases therfore the focus of treatment should be to maximize normal organ function and alleviate symptoms.
Why Are The Brain, Kidneys, Nerves, Retinas
and Muscles So Vulnerable?
Their tissues tend to have a high demand
for energy. The diseased cells of the eyes and muscles cannot be replaced by healthier neighbouring cells which is a
process that occurs in tissue with cellular turnover such as the skin or mucosa.
Nerves
Nerve cells are extremely active metabolically
and require a tremendous amount of energy to maintain the electrochemical gradient necessary for nerve transmission. Neuropathy
results in temperature instability, inappropriate sweating resulting in heat stroke, bladder dysfunction, gastrointestinal
dysmotility, loss of reflexes, weakness, or neuropathic pain.
