RRC ID 46656
Author Mun JY, Lee TH, Kim JH, Yoo BH, Bahk YY, Koo HS, Han SS.
Title Caenorhabditis elegans mitofilin homologs control the morphology of mitochondrial cristae and influence reproduction and physiology.
Journal J Cell Physiol
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.
Volume 224(3)
Pages 748-56
Published 2010-9-1
DOI 10.1002/jcp.22177
PMID 20578245
MeSH 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
IF 5.546
Times Cited 57
C.elegans tm1730 tm2366