A quantitative fluorescence-based approach to study mitochondrial protein import.

Mitochondria play central roles in cellular energy production and metabolism. Most proteins required to carry out these functions are synthesized in the cytosol and imported into mitochondria. A growing number of metabolic disorders arising from mitochondrial dysfunction can be traced to errors in mitochondrial protein import.

Defective Mitochondrial Cardiolipin Remodeling Dampens HIF-1α Expression in Hypoxia.

Mitochondria fulfill vital metabolic functions and act as crucial cellular signaling hubs, integrating their metabolic status into the cellular context. Here, we show that defective cardiolipin remodeling, upon loss of the cardiolipin acyl transferase tafazzin, decreases HIF-1α signaling in hypoxia. Tafazzin deficiency does not affect posttranslational HIF-1α regulation but rather HIF-1α gene expression, a dysfunction recapitulated in iPSC-derived cardiomyocytes from Barth syndrome patients with tafazzin deficiency.

Recognition and ER Quality Control of Misfolded Formylglycine-Generating Enzyme by Protein Disulfide Isomerase.

Multiple sulfatase deficiency (MSD) is a fatal, inherited lysosomal storage disorder characterized by reduced activities of all sulfatases in patients. Sulfatases require a unique post-translational modification of an active-site cysteine to formylglycine that is catalyzed by the formylglycine-generating enzyme (FGE). FGE mutations that affect intracellular protein stability determine residual enzyme activity and disease severity in MSD patients.

Presequence recognition by the tom40 channel contributes to precursor translocation into the mitochondrial matrix.

More than 70% of mitochondrial proteins utilize N-terminal presequences as targeting signals. Presequence interactions with redundant cytosolic receptor domains of the translocase of the outer mitochondrial membrane (TOM) are well established. However, after the presequence enters the protein-conducting Tom40 channel, the recognition events that occur at the trans side leading up to the engagement of the presequence with inner membrane-bound receptors are less well defined.

Occlusal contact patterns--population-based data.

The reconstruction of functionally appropriate contacts between antagonistic teeth substantially determines the quality of prosthetic-restorative work. In a population-based sample comprising 2597 subjects, static occlusal contacts were registered and analysed using the GEDAS (Greifswald Digital Analyzing System), which digitally represents the contact point situation by means of silicone bite impressions. The number of contacting teeth is approximately equal on the Left- and right-hand side amounting to 8.

Partial- or full-mouth examination assessing the dental and prosthetic status among elderly individuals.

The aim of this study was to determine whether half-mouth examinations accurately reflect the dental and prosthetic status of the entire mouth. Samples of 1,830 adults aged 55- 79 years were examined. The rate of agreement between half- and full-mouth examinations was estimated using weighted and unweighted Kappa values comparing findings of each tooth bilaterally.

AP-1A and AP-3A lysosomal sorting functions.

Heterotetrameric adaptor-protein complexes AP-1A and AP-3A mediate protein sorting in post-Golgi vesicular transport. AP-1A and AP-3A have been localized to the trans-Golgi network, indicating a function in protein sorting at this compartment. AP-3A appears to mediate trans-Golgi network-to-lysosome and also endosome-to-lysosome protein sorting.