• To understand mitochondrial pathways in health and disease, it is crucial to have an accurate inventory of the organelle's protein components. (nih.gov)
  • This motif is conspicuously absent, however, in all but one of the TA protein subunits of the translocon at the outer membrane of mitochondria (TOM), suggesting that these two groups of proteins utilize distinct biogenetic pathways. (frontiersin.org)
  • We compare UPR mt with the extensively characterized cytosolic heat shock response (HSR) and the unfolded protein response in endoplasmic reticulum (UPR ER ), and discuss the current knowledge about UPR mt signaling pathways as well as their potential involvement in physiology. (biologists.org)
  • Maintenance of protein homeostasis, also known as 'proteostasis', which refers to all the cellular pathways that govern the production, folding and degradation of proteins, is essential for cellular and organismal functionality and survival. (biologists.org)
  • Collectively, their work suggests that mitochondrial protein hyperacetylation may promote the metabolic defects seen in heart failure. (medindia.net)
  • A study performed at IRB Barcelona supports the notion that mitochondrial defects underlie a set of diseases of unknown origin that involve chronic muscle inflammation. (irbbarcelona.org)
  • Mitochondrial content is increased in CSB-deficient cells, whereas autophagy is down-regulated, presumably as a result of defects in the recruitment of P62 and mitochondrial ubiquitination. (sigmaaldrich.com)
  • Therefore, evidence is provided that on the one hand questions our current understanding of the UPRmt signalling in mammals, but on the other hand identifies CLPP as novel modulator of mitochondrial translation, which might be used for the development of new therapeutic approaches against translational defects in mitochondrial disease. (uni-koeln.de)
  • In order to address these open issues, permanent cell lines were generated within the frame of the present thesis in which the expression of single proteins implicated in mitochondrial import can be inhibited via RNA interference (RNAi). (hu-berlin.de)