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Importing Mitochondrial Proteins: Machineries and Mechanisms
Recent insights into the importing and sorting of mitochondrial proteins and their contributions to mitochondrial biogenesis are discussed. Expand
UCP2 regulates energy metabolism and differentiation potential of human pluripotent stem cells
Overall, hPSCs contain active mitochondria and require UCP2 repression for full differentiation potential, and have functional respiratory complexes that are able to consume O2 at maximal capacity. Expand
Presenilins are enriched in endoplasmic reticulum membranes associated with mitochondria.
Using three complementary approaches, subcellular fractionation, gamma-secretase activity assays, and immunocytochemistry, it is shown that presenilins are highly enriched in a subcompartment of the endoplasmic reticulum that is associated with mitochondria and that forms a physical bridge between the two organelles, called endoplasic Reticulum-mitochondria-associated membranes. Expand
Import of mitochondrial carriers mediated by essential proteins of the intermembrane space.
Two essential proteins, Tim10p and Tim12p, were shown to mediate import of multispanning carriers into the inner membrane of the mitochondrion and may function as intermembrane space chaperones for the highly insoluble carriers. Expand
PNPASE Regulates RNA Import into Mitochondria
PNPASE RNA processing and import activities were separable and a mitochondrial RNA targeting signal was isolated that enabled RNA import in a PNPASE-dependent manner, and these data strongly support an unanticipated role for P NPASE in mediating the translocation of RNAs into mitochondria. Expand
Tim9p, an essential partner subunit of Tim10p for the import of mitochondrial carrier proteins
Tim9p is a new subunit of the TIM machinery that guides hydrophobic inner membrane proteins across the aqueous intermembrane space and co‐immunoprecipitated with each other. Expand
The Tim9p–Tim10p complex binds to the transmembrane domains of the ADP/ATP carrier
Results from inductively coupled plasma–mass spectrometry studies failed to detect zinc in the Tim9p–Tim10p complex, but the cysteine residues seemingly formed disulfide linkages and may prevent aggregation of the unfolded carrier proteins in the aqueous intermembrane space. Expand
Human deafness dystonia syndrome is a mitochondrial disease.
It is shown that DDP is a mitochondrial protein and similar to five small proteins of the yeast mitochondrial intermembrane space that mediate the import of metabolite transporters from the cytoplasm into the mitochondrial inner membrane and interact structurally and functionally with Tim8p and Tim13p. Expand
New developments in mitochondrial assembly.
  • C. Koehler
  • Biology, Medicine
  • Annual review of cell and developmental biology
  • 8 October 2004
The mitochondrion has developed an elaborate translocation system for the import of nuclear-coded proteins and the export of proteins coded on the mitochondrial genome that mediate the export and assembly of proteins from within the matrix. Expand
Cardiolipin defines the interactome of the major ADP/ATP carrier protein of the mitochondrial inner membrane
It is demonstrated that yeast AAC2 physically participates in several protein complexes of distinct size and composition, including the respiratory supercomplex and several smaller AAC2-containing complexes, including other members of the mitochondrial carrier family, are identified here. Expand