Cristae formation

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R-HSA-8949613
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Pathway
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Homo sapiens
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Cristae are invaginations of the inner mitochondrial membrane that extend into the matrix and are lined with cytochrome complexes and F1Fo ATP synthase complexes. Cristae increase the surface area of the inner membranes allowing greater numbers of respiratory complexes. Cristae are also believed to serve as "proton pockets" to generate localized regions of higher membrane potential. The steps in the biogenesis of cristae are not yet completely elucidated (reviewed in Zick et al. 2009) but the formation of the Mitochondrial Contact Site and Cristae Organizing System (MICOS, formerly also known as MINOS, reviewed in Rampelt et al. 2016, Kozjak-Pavlovic 2016, van der Laan et al. 2016) and localized concentrations of cardiolipin are known to define the inward curvature of the inner membrane at the bases of cristae. MICOS also links these regions of the inner membrane with complexes (the SAM complex and, in fungi, the TOM complex) embedded in the outer membrane. CHCHD3 (MIC19) and IMMT (MIC60) subunits of MICOS also interact with OPA1 at the inner membrane (Darshi et al. 2011, Glytsou et al. 2016).
Formation of dimers or oligomers of the F1Fo ATP synthase complex causes extreme curvature of the inner membrane at the apices of cristae (reviewed in Seelert and Dencher 2011, Habersetzer et al. 2013). Defects in either MICOS or F1Fo ATP synthase oligomerization produce abnormal mitochondrial morphologies.

Literature References
PubMed ID Title Journal Year
22664329 ATP synthase oligomerization: from the enzyme models to the mitochondrial morphology

Habersetzer, J, Ziani, W, Larrieu, I, Stines-Chaumeil, C, Giraud, MF, Brèthes, D, Dautant, A, Paumard, P

Int. J. Biochem. Cell Biol. 2013
27245231 The MICOS complex of human mitochondria

Kozjak-Pavlovic, V

Cell Tissue Res. 2016
27614134 Role of the mitochondrial contact site and cristae organizing system in membrane architecture and dynamics

Rampelt, H, Zerbes, RM, van der Laan, M, Pfanner, N

Biochim. Biophys. Acta 2016
21081504 ChChd3, an inner mitochondrial membrane protein, is essential for maintaining crista integrity and mitochondrial function

Darshi, M, Mendiola, VL, Mackey, MR, Murphy, AN, Koller, A, Perkins, GA, Ellisman, MH, Taylor, SS

J. Biol. Chem. 2011
18620004 Cristae formation-linking ultrastructure and function of mitochondria

Zick, M, Rabl, R, Reichert, AS

Biochim. Biophys. Acta 2009
21679683 ATP synthase superassemblies in animals and plants: two or more are better

Seelert, H, Dencher, NA

Biochim. Biophys. Acta 2011
27062547 Mitochondrial contact site and cristae organizing system

van der Laan, M, Horvath, SE, Pfanner, N

Curr. Opin. Cell Biol. 2016
27974214 Optic Atrophy 1 Is Epistatic to the Core MICOS Component MIC60 in Mitochondrial Cristae Shape Control

Glytsou, C, Calvo, E, Cogliati, S, Mehrotra, A, Anastasia, I, Rigoni, G, Raimondi, A, Shintani, N, Loureiro, M, Vázquez, J, Pellegrini, L, Enríquez, JA, Scorrano, L, Soriano, ME

Cell Rep 2016
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