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PMID:20578245

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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

Significance

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


See also

References

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