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Home > B. Cellular pathology > mitochondria


Sunday 13 July 2003

Mitochondrial structure

- inner mitochondrial membrane

Mitochondrial genome

- structure
- maitenance
- replication
- mitochondrial transcription
- mitochondrial traduction
- elongation
- termination
- co-traductional phenomenons

Mitochondrial functions

- energy production/ROS (rective oxygen species)
- programmed cell death (apoptosis)
- calcium homeostasis
- gluconeogenesis
- urea cycle
- lipid synthesis
- fatty acid oxidation

Mitochondria and apoptosis

- Involvement of mitochondria in apotposis is regulated by proteins of the Bcl-2 (B-cell lymphocytic-leukaemia proto-oncogene 2 or BCL2) family. These proteins act by altering the properties of mitochondrial membranes to facilitate the release of apoptogenic proteins like cytochrome c and Smac/Diablo that, once released into the cytosol, are crucial for activating the caspase cascade of cell degradation.

- CL (cardiolipin), an unusual membrane lipid that is predominantly present in mitochondria, is required for the action of major pro-apoptotic proteins like Bid and Bax. Bid may be involved in the metabolic cycle of CL re-modelling.

Mitochondrial dynamics

Mitochondrial organelles constantly fuse and divide, and are actively transported to specific subcellular locations. These dynamic processes are essential for mammalian development, and defects lead to neurodegenerative diseases.


- mitochondrial lesions
- mitochondrial diseases

See also

- mitochondrial fusion
- mitochondrial genesis
- mitochondrial death
- mitochondria and cellular death (mitochondria and apoptosis)


- Modica-Napolitano JS, Kulawiec M, Singh KK. Mitochondria and human cancer. Curr Mol Med. 2007 Feb;7(1):121-31. PMID: 17311537

- Boldogh IR, Pon LA. Mitochondria on the move. Trends Cell Biol. 2007 Oct;17(10):502-10. PMID: 17804238

- Scherz-Shouval R, Elazar Z. ROS, mitochondria and the regulation of autophagy. Trends Cell Biol. 2007 Sep;17(9):422-7. PMID: 17804237

- Baker MJ, Frazier AE, Gulbis JM, Ryan MT. Mitochondrial protein-import machinery: correlating structure with function. Trends Cell Biol. 2007 Sep;17(9):456-64. PMID: 17825565

- Pizzo P, Pozzan T. Mitochondria-endoplasmic reticulum choreography: structure and signaling dynamics.Trends Cell Biol. 2007 Oct;17(10):511-7. PMID: 17851078

- Detmer SA, Chan DC. Functions and dysfunctions of mitochondrial dynamics. Nat Rev Mol Cell Biol. 2007 Nov;8(11):870-9. PMID: 17928812

- Meeusen SL, Nunnari J. How mitochondria fuse. Curr Opin Cell Biol. 2005 Aug;17(4):389-94. PMID: 15975776

- Jensen RE. Control of mitochondrial shape. Curr Opin Cell Biol. 2005 Aug;17(4):384-8. PMID: 15975781

- Mattson MP, Kroemer G. Mitochondria in cell death: novel targets for neuroprotection and cardioprotection. Trends Mol Med. 2003 May;9(5):196-205. PMID: 12763524

- Chacinska A, Pfanner N, Meisinger C. How mitochondria import hydrophilic and hydrophobic proteins. Trends Cell Biol. 2002 Jul;12(7):299-303. PMID: 12185844

- Ravagnan L, Roumier T, Kroemer G. Mitochondria, the killer organelles and their weapons. J Cell Physiol. 2002 Aug;192(2):131-7. PMID: 12115719

- Cohen I, Castedo M, Kroemer G. Tantalizing Thanatos: unexpected links in death pathways. Trends Cell Biol. 2002 Jul;12(7):293-5. PMID: 12185842

- Desagher S, Martinou JC. Mitochondria as the central control point of apoptosis. Trends Cell Biol. 2000 Sep ;10(9):369-77. PMID : 10932094