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Mitochondrial disease - Wikipedia, the free encyclopediaMitochondrial disease From Wikipedia, the free encyclopedia Jump to: navigation, search Mitochondrial disease Classification and external resources Simplified structure of a typical mitochondrion DiseasesDB28840 MeSHD028361 Mitochondrial diseases are a group of disorders relating to the mitochondria, the organelles that are the "powerhouses" of the eukaryotic cells that comprise higher-order lifeforms (including humans). The mitochondria convert the energy of food molecules into the ATP that powers most cell functions. Mitochondrial diseases comprise those disorders that in one way or another affect the function of the mitochondria and/or are due to mitochondrial DNA. Mitochondrial diseases take on unique characteristics both because of the way the diseases are often inherited and because mitochondria are so critical to cell function. The subclass of these diseases that have neuromuscular disease symptoms are often referred to as a mitochondrial myopathy. Contents [hide] 1 Mitochondrial inheritance 2 Defects and symptoms 3 Types 4 Treatment 5 References 6 External links [edit] Mitochondrial inheritance Mitochondrial inheritance behaves differently from autosomal and sex-linked inheritance. Nuclear DNA has two copies per cell (except for sperm and egg cells). One copy is inherited from the father and the other from the mother. Mitochondria, however, contain their own DNA, and contain typically from five to ten copies (see Heteroplasmy), all inherited from the mother (for more detailed inheritance patterns, see Human mitochondrial genetics). When the mitochondria divides, the copies of DNA present are divided randomly between the two new mitochondria, and then those new mitochondria make more copies. As a result, if only a few of the DNA copies inherited from the mother are defective, mitochondrial division may cause most of the defective copies to end up in just one of the new mito...