Mitochondrial.org

Below are basic facts about the mitochondria

Mitochondria Facts

· Mitochondria is the site of respiration in the cell

· Mitochondria is thought to have evolved from symbiosis between two eukaryote cells

Mitochondria Description

· The four main parts of mitochondria are the outer membrane, intermembrane space, inner membrane, and the matrix

· The inner membrane folds into cristae in order to increase surface area

· The mitochondria divides independently of the cell

Mitochondria Structure

· Mitochondrial dysfunction is the primary problem in many diseases including diabetes, infantile cardiomyopathy, Friedreich ataxia, nonketotic hyperglycinemia, and Luft disease.  It also plays a role in Alzheimer’s, Parkinson’s, autism, and the aging process.

· The main problems associated with mitochondrial diseases are free radical formation, low energy, and lactic acidosis

Mitochondrial Diseases

· The chief function of mitochondria is to create energy by the process of aerobic respiration.  Glucose is broken down in the cell’s cytoplasm to form pyruvic acid or pyruvate, which is transported into the mitochondrion.

· In a series of reactions, part of which is called Krebs Cycle, pyruvate reacts with water to produce carbon dioxide and ten protons or hydrogen atoms.  These protons are transported on special carrier molecules called coenzymes to the cristae, where they are donated to the electron transport chain.

· Mitochondria also metabolize amino acids, fatty acids, and steroids

· Mitochondria create more than 90% of the energy needed by the body to support growth.  Mitochondria dysfunction leads to less energy generation by the cell and may cause serious injury or death.

Mitochondria Function

· Mitochondrial DNA (mtDNA) has been mapped completely

· MtDNA contains 16,569 base pairs

· The DNA encodes 13 mitochondrial subunits, 22 transfer ribonucleic acids (tRNAs), 2 ribosomal ribonucleic acids (rRNAs), and polypetides.  These are found in mitochondrial complexes I, III, IV, and V.

· mtDNA is inherited maternally; therefore, the only way the mtDNA can change is by sequential accumulation of mutations along the maternal lineage.

Mitochondrial DNA

References:        http://www.mitoresearch.org/

                          http://www.neuro.wustl.edu/neuromuscular/mitosyn.html