Mitochondrial Mythology Research Paper
NTRODUCTION :
Ethymology : "myo" means muscle and "pathos" means disease.
The term "mitochondrial disease" was introduced in 1962 by a group of scientifics, including the endocrinologist Rolf Luft, the biochemist Lars Ernster, and the morphologist Björn
Afzelius. Their investigation were based on a woman with a severe hypermetabolism. They worked on three points : abnormal mitochondria in muscle, a great correlation between biochemical abnormalities and clinical features (uncontrolled muscle metabolism), and biochemicaldocumentation of "loose coupling" of oxidation and phosphorylation in isolated muscle mitochondria.
In 1985, a general biochemical classification of the mitochondrial diseases was proposed, based on defects of substrate transport …show more content…
The mitochondria produce ATP, and this ATP provides the main source of energy for muscle cell contraction and nerve celle firing. So, if there is a mitochondrial defect, muscle cells and nerve cells are sensibly touched : it provoke the main symptoms of mitochondrial myopathies. A mitochondrial disease can shut down some or all the mitochondria, cutting off this essential energy supply. The most of mitochondrial myopathies starts before the age of 20 and often with muscular weakness. After that, the symptoms may be very different or the same but with different potential causes, and the severity of the disease too. It depends on many factors : genetic, environmental and physiologic factors. This sort of disease is progressive and the rate of its progression is unpredictable. In the first part, we will present the two main terms of this subject : the mitochondrion and the adenosine triphosphate. Then, we will present the causes of a mitochondrial myopathy, which are a genetic cause and a trouble in the respiratory chain. Then we all present the mitochondrial myopathies and we will finish by explaining the different possibilities
Ethymology : "myo" means muscle and "pathos" means disease.
The term "mitochondrial disease" was introduced in 1962 by a group of scientifics, including the endocrinologist Rolf Luft, the biochemist Lars Ernster, and the morphologist Björn
Afzelius. Their investigation were based on a woman with a severe hypermetabolism. They worked on three points : abnormal mitochondria in muscle, a great correlation between biochemical abnormalities and clinical features (uncontrolled muscle metabolism), and biochemicaldocumentation of "loose coupling" of oxidation and phosphorylation in isolated muscle mitochondria.
In 1985, a general biochemical classification of the mitochondrial diseases was proposed, based on defects of substrate transport …show more content…
The mitochondria produce ATP, and this ATP provides the main source of energy for muscle cell contraction and nerve celle firing. So, if there is a mitochondrial defect, muscle cells and nerve cells are sensibly touched : it provoke the main symptoms of mitochondrial myopathies. A mitochondrial disease can shut down some or all the mitochondria, cutting off this essential energy supply. The most of mitochondrial myopathies starts before the age of 20 and often with muscular weakness. After that, the symptoms may be very different or the same but with different potential causes, and the severity of the disease too. It depends on many factors : genetic, environmental and physiologic factors. This sort of disease is progressive and the rate of its progression is unpredictable. In the first part, we will present the two main terms of this subject : the mitochondrion and the adenosine triphosphate. Then, we will present the causes of a mitochondrial myopathy, which are a genetic cause and a trouble in the respiratory chain. Then we all present the mitochondrial myopathies and we will finish by explaining the different possibilities