mitochondrially encoded cytochrome b
Last updated 18 Aug 2020
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The MT-CYB gene provides instructions for making a protein called cytochrome b. This protein plays a key role in structures called mitochondria, which convert the energy from food into a form that cells can use. Cytochrome b is one of 11 components of a group of proteins called complex III. In mitochondria, complex III performs one step of a process known as oxidative phosphorylation, in which oxygen and simple sugars are used to create adenosine triphosphate (ATP), the cell's main energy source. During oxidative phosphorylation, the protein complexes, including complex III, drive the production of ATP through a step-by-step transfer of negatively charged particles called electrons. Cytochrome b is involved in the transfer of these particles through complex III.
Normal function
The MT-CYB gene provides instructions for making a protein called cytochrome b. This protein plays a key role in structures called mitochondria, which convert the energy from food into a form that cells can use. Cytochrome b is one of 11 components of a group of proteins called complex III. In mitochondria, complex III performs one step of a process known as oxidative phosphorylation, in which oxygen and simple sugars are used to create adenosine triphosphate (ATP), the cell's main energy source. During oxidative phosphorylation, the protein complexes, including complex III, drive the production of ATP through a step-by-step transfer of negatively charged particles called electrons. Cytochrome b is involved in the transfer of these particles through complex III.
Although most DNA is packaged in chromosomes within the nucleus (nuclear DNA), mitochondria also have a small amount of their own DNA, called mitochondrial DNA (mtDNA). This type of DNA contains many genes essential for normal mitochondrial function. Cytochrome b is the only component of complex III that is produced from a gene found in mitochondrial DNA.
Conditions referring to this gene or chromosome
Other names
COB
CYTB
cytochrome b
cytochrome b (mitochondrion) [Homo sapiens]
MTCYB
UQCR3
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Further reading and references
- mitochondrially encoded cytochrome b; MedlinePlus, National Library of Medicine.
- Andreu AL, Hanna MG, Reichmann H, Bruno C, Penn AS, Tanji K, Pallotti F, Iwata S, Bonilla E, Lach B, Morgan-Hughes J, DiMauro S. Exercise intolerance due to mutations in the cytochrome b gene of mitochondrial DNA. N Engl J Med. 1999 Sep 30;341(14):1037-44. doi: 10.1056/NEJM199909303411404.
- Blakely EL, Mitchell AL, Fisher N, Meunier B, Nijtmans LG, Schaefer AM, Jackson MJ, Turnbull DM, Taylor RW. A mitochondrial cytochrome b mutation causing severe respiratory chain enzyme deficiency in humans and yeast. FEBS J. 2005 Jul;272(14):3583-92. doi: 10.1111/j.1742-4658.2005.04779.x.
- Gil Borlado MC, Moreno Lastres D, Gonzalez Hoyuela M, Moran M, Blazquez A, Pello R, Marin Buera L, Gabaldon T, Garcia Penas JJ, Martin MA, Arenas J, Ugalde C. Impact of the mitochondrial genetic background in complex III deficiency. PLoS One. 2010 Sep 17;5(9):e12801. doi: 10.1371/journal.pone.0012801.
- Meunier B, Fisher N, Ransac S, Mazat JP, Brasseur G. Respiratory complex III dysfunction in humans and the use of yeast as a model organism to study mitochondrial myopathy and associated diseases. Biochim Biophys Acta. 2013 Nov-Dec;1827(11-12):1346-61. doi: 10.1016/j.bbabio.2012.11.015. Epub 2012 Dec 5.
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