PMID:20578245

Citation	Mun, JY, Lee, TH, Kim, JH, Yoo, BH, Bahk, YY, Koo, HS and Han, SS (2010) Caenorhabditis elegans mitofilin homologs control the morphology of mitochondrial cristae and influence reproduction and physiology. J. Cell. Physiol. 224:748-56
Abstract	Human mitofilin is a mitochondrial protein that controls cristae formation. Here, we investigated the role of the Caenorhabditis elegans mitofilin homologs, IMMT-1 and -2, in reproduction, physiology, and mitochondrial cristae formation. Mutation of either immt-1 or immt-2 produced defects in germline development and egg-laying. These defects were exacerbated by the double mutation, which greatly reduced motility, increased levels of reactive oxygen species, decreased mitochondrial mass, and imparted resistance to oxidative stress. Cryo-electron microscopy and electron tomography revealed that each of the single mutations resulted in curved and stacked mitochondrial crista tubules as well as a reduced number of crista junctions. The immt-2 mutation was also associated with the presence of outer mitochondrial membrane pores, which were larger in the double mutant. IMMT-1 and IMMT-2 proteins were localized to the inner mitochondrial membrane, as seen by immunoelectron microscopy, and they behaved as oligomers or large complexes with F(1)F(0) ATP synthase in native polyacrylamide gel electrophoresis. These findings suggest that the two C. elegans mitofilin isoforms have non-overlapping functions in controlling mitochondrial cristae formation.
Links	PubMed Online version:10.1002/jcp.22177
Keywords	Animals; Caenorhabditis elegans/cytology; Caenorhabditis elegans/genetics; Caenorhabditis elegans/physiology; Caenorhabditis elegans Proteins/genetics; Caenorhabditis elegans Proteins/metabolism; Humans; Mitochondria/metabolism; Mitochondria/ultrastructure; Mitochondrial Proteins/genetics; Mitochondrial Proteins/metabolism; Mutation; Protein Isoforms/genetics; Protein Isoforms/metabolism; Reactive Oxygen Species/metabolism
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Annotations

Gene product	Qualifier	GO Term	Evidence Code	with/from	Aspect	Extension	Notes	Status
CAEEL:IMMT1	part_of	GO:0005753: mitochondrial proton-transporting ATP synthase complex	ECO:0000314: direct assay evidence used in manual assertion		C		Seeded From UniProt	complete
CAEEL:IMMT1	located_in	GO:0005743: mitochondrial inner membrane	ECO:0000314: direct assay evidence used in manual assertion		C		Seeded From UniProt	complete
CAEEL:IMMT1	involved_in	GO:0070584: mitochondrion morphogenesis	ECO:0000315: mutant phenotype evidence used in manual assertion	WB:WBVar00250697	P		Seeded From UniProt	complete
CAEEL:IMMT1	involved_in	GO:0042407: cristae formation	ECO:0000315: mutant phenotype evidence used in manual assertion	WB:WBVar00250697	P		Seeded From UniProt	complete
CAEEL:IMMT1	involved_in	GO:0000302: response to reactive oxygen species	ECO:0000315: mutant phenotype evidence used in manual assertion	WB:WBVar00250697	P		Seeded From UniProt	complete
CAEEL:IMMT2	part_of	GO:0005753: mitochondrial proton-transporting ATP synthase complex	ECO:0000314: direct assay evidence used in manual assertion		C		Seeded From UniProt	complete
CAEEL:IMMT2	located_in	GO:0005743: mitochondrial inner membrane	ECO:0000314: direct assay evidence used in manual assertion		C		Seeded From UniProt	complete
CAEEL:IMMT2	involved_in	GO:0070584: mitochondrion morphogenesis	ECO:0000315: mutant phenotype evidence used in manual assertion	WB:WBVar00251261	P		Seeded From UniProt	complete
CAEEL:IMMT2	involved_in	GO:0042407: cristae formation	ECO:0000315: mutant phenotype evidence used in manual assertion	WB:WBVar00251261	P		Seeded From UniProt	complete
CAEEL:IMMT2	involved_in	GO:0000302: response to reactive oxygen species	ECO:0000315: mutant phenotype evidence used in manual assertion	WB:WBVar00251261	P		Seeded From UniProt	complete

References

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