A mitochondrion (/ˌmaɪtəˈkɒndriən/,[1] plural mitochondria) is a double membrane-bound organelle found in most eukaryotic organisms. Mitochondria generate most of the cell’s supply of adenosine triphosphate (ATP), used as a source of chemical energy.[2] Mitochondria were first discovered by Kolliker (1880 CE) in the voluntary muscles of insects. A mitochondrion is nicknamed the “powerhouse of the cell”, first coined by Philip Siekevitz in a 1957 article of the same name.[3]Some cells in some multicellular organisms lack mitochondria (for example, mature ma
mmalian red blood cells). A large number of unicellular organisms, such as microsporidia, parabasalids, and diplomonads, have reduced or transformed their mitochondria into other structures.[4] One eukaryote, Monocercomonoides, is known to have completely lost its mitochondria,[5] and one multicellular organism, Henneguya salminicola, is known to have retained mitochondrion-related organelles in association with a complete loss of their mitochondrial genome.[5][6][7]
Mitochondria are commonly between 0.75 and 3 μm² in area[8] but they vary considerably in size and structure. Unless specifically stained, they are not visible. In addition to supplying cellular energy, mitochondria are involved in other tasks, such as signaling, cellular differentiation, and cell death, as well as maintaining control of the cell cycle and cell growth.[9] Mitochondrial biogenesis is in turn temporally coordinated with these cellular processes.[10][11] Mitochondria have been implicated in several human diseases and conditions, such as mitochondrial disorders,[12] cardiac dysfunction,[13] heart failure[14] and autism.[15]
The number of mitochondria in a cell can vary widely by organism, tissue, and cell type. A mature red blood cell has no mitochondria,[16] whereas a liver cell can have more than 2000.[17][18] The mitochondrion is composed of compartments that carry out specialized functions. These compartments or regions include the outer membrane, intermembrane space, inner membrane, cristae and matrix.
Although most of a cell’s DNA is contained in the cell nucleus, the mitochondrion has its own genome (“mitogenome”) that is substantially similar to bacterial genomes.[19] Mitochondrial proteins (proteins transcribed from mitochondrial DNA) vary depending on the tissue and the species. In humans, 615 distinct types of proteins have been identified from cardiac mitochondria,[20] whereas in rats, 940 proteins have been reported.[21] The mitochondrial proteome is thought to be dynamically regulated.[22]
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