MITOCHONDRIAL DISEASES
MITOCHONDRIAL DISEASES
The mitochondrion is the energy manufacturer of the cell. It is equivalent to the engine of a car. These tiny biological machines combine the food we eat with oxygen to produce energy to keep our bodies going. The energy produced by the mitochondria is stored in the form of a chemical called Adenosine triphosphate (ATP).
The mitochondria is involved in making of steroid hormones and DNA. Each cell in the body contains an
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Urine amino acids and plasma amino acids (elevated levels of the amino acids leucine, isoleucine and valine).
PREVENTION:
Genetic counseling is suggested for prospective parents with a family history of maple syrup.
TREATMENT:
For long-range treatments, it includes special diet, which involves strict adherence to prevent neurological damage. Also, treatment for the acute episode: peritoneal dialysis can be used to remove the high levels of amino acids. Acute acidosis is treated to restore normal pH.
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