The mitochondrial inner membrane protein mitofilin exists as a complex with SAM50, metaxins 1 and 2, coiled-coil-helix coiled-coil-helix domain-containing protein 3 and 6 and DnaJC11

FEBS Lett. 2007 Jul 24;581(18):3545-9. doi: 10.1016/j.febslet.2007.06.052. Epub 2007 Jun 27.


A monoclonal antibody (mAb) has been produced which reacts with human mitofilin, a mitochondrial inner membrane protein. This mAb immunocaptures its target protein in association with six other proteins, metaxins 1 and 2, SAM50, CHCHD3, CHCHD6 and DnaJC11, respectively. The first three are outer membrane proteins, CHCHD3 has been assigned to the matrix space, and the other two proteins have not been described in mitochondria previously. The functional role of this new complex is uncertain. However, a role in protein import related to maintenance of mitochondrial structure is suggested as mitofilin helps regulate mitochondrial morphology and at least four of the associated proteins (metaxins 1 and 2, SAM50 and CHCHD3) have been implicated in protein import, while DnaJC11 is a chaperone-like protein that may have a similar role.

MeSH terms

  • Animals
  • Humans
  • Membrane Proteins / metabolism*
  • Mice
  • Mitochondrial Membranes / metabolism
  • Mitochondrial Proteins / metabolism*
  • Multiprotein Complexes / chemistry
  • Multiprotein Complexes / metabolism
  • Muscle Proteins / metabolism*
  • Myocardium / metabolism
  • Protein Binding
  • Proteins / metabolism*
  • Tandem Mass Spectrometry


  • CHCHD3 protein, human
  • CHCHD6 protein, human
  • DNAJC11 protein, human
  • IMMT protein, human
  • MTX1 protein, human
  • Membrane Proteins
  • Mitochondrial Proteins
  • Multiprotein Complexes
  • Muscle Proteins
  • Proteins
  • SAMM50 protein, human
  • metaxin 2 protein, human