Mitofilin complexes: conserved organizers of mitochondrial membrane architecture

Abstract: Mitofilin proteins are crucial organizers of mitochondrial architecture. They are located in the inner mitochondrial membrane and interact with several protein complexes of the outer membrane, thereby generating contact sites between the two membrane systems of mitochondria. Within the inner membrane, mitofilins are part of hetero-oligomeric protein complexes that have been termed the mitochondrial inner membrane organizing system (MINOS). MINOS integrity is required for the maintenance of the characteristic morphology of the inner mitochondrial membrane, with an inner boundary region closely apposed to the outer membrane and cristae membranes, which form large tubular invaginations that protrude into the mitochondrial matrix and harbor the enzyme complexes of the oxidative phosphorylation machinery. MINOS deficiency comes along with a loss of crista junction structures and the detachment of cristae from the inner boundary membrane. MINOS has been conserved in evolution from unicellular eukaryotes to humans, where alterations of MINOS subunits are associated with multiple pathological conditions.

Location
Deutsche Nationalbibliothek Frankfurt am Main
Extent
Online-Ressource
Language
Englisch

Bibliographic citation
Mitofilin complexes: conserved organizers of mitochondrial membrane architecture ; volume:393 ; number:11 ; year:2012 ; pages:1247-1261
Biological chemistry ; 393, Heft 11 (2012), 1247-1261

Creator
Zerbes, Ralf Michael
van der Klei, Ida J.
Veenhuis, Marten
Pfanner, Nikolaus
van der Laan, Martin
Bohnert, Maria

DOI
10.1515/hsz-2012-0239
URN
urn:nbn:de:101:1-2408051618356.519089006173
Rights
Open Access; Der Zugriff auf das Objekt ist unbeschränkt möglich.
Last update
14.08.2025, 10:44 AM CEST

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Associated

  • Zerbes, Ralf Michael
  • van der Klei, Ida J.
  • Veenhuis, Marten
  • Pfanner, Nikolaus
  • van der Laan, Martin
  • Bohnert, Maria

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