Developmental Cell
Volume 21, Issue 4, 18 October 2011, Pages 694-707
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Article
Dual Role of Mitofilin in Mitochondrial Membrane Organization and Protein Biogenesis

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Summary

The mitochondrial inner membrane consists of two domains, inner boundary membrane and cristae membrane that are connected by crista junctions. Mitofilin/Fcj1 was reported to be involved in formation of crista junctions, however, different views exist on its function and possible partner proteins. We report that mitofilin plays a dual role. Mitofilin is part of a large inner membrane complex, and we identify five partner proteins as constituents of the mitochondrial inner membrane organizing system (MINOS) that is required for keeping cristae membranes connected to the inner boundary membrane. Additionally, mitofilin is coupled to the outer membrane and promotes protein import via the mitochondrial intermembrane space assembly pathway. Our findings indicate that mitofilin is a central component of MINOS and functions as a multifunctional regulator of mitochondrial architecture and protein biogenesis.

Highlights

► Mitofilin/Fcj1 is part of a large protein complex of the mitochondrial inner membrane ► This MINOS complex regulates cristae morphology ► In MINOS mutants cristae are released from the inner boundary membrane (IBM) ► Mitofilin interacts with the outer membrane and promotes protein import

Cited by (0)

13

These authors contributed equally to this work

14

Present address: Medical Centre for Postgraduate Education, 01-813 Warsaw, Poland

15

Present address: Max Planck Institute for Biology of Ageing, 50931 Cologne, Germany

16

Present address: European Commission, Joint Research Centre, Institute for Reference Materials and Measurements, 2440 Geel, Belgium