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Biogenesis of Mitochondrial Proteins
2012Depending on the organism, mitochondria consist approximately of 500-1,400 different proteins. By far most of these proteins are encoded by nuclear genes and synthesized on cytosolic ribosomes. Targeting signals direct these proteins into mitochondria and there to their respective subcompartment: the outer membrane, the intermembrane space (IMS), the ...
Johannes M, Herrmann +3 more
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1991
A dynamic picture of the mitochondrial protein import pathway is emerging, with conformational alteration a critical feature both preceding and following membrane translocation. The mediators of these steps of conformational alteration, as well as steps of recognition, translocation, and proteolytic cleavage, appear to be proteins. Using powerful tools
A L, Horwich +3 more
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A dynamic picture of the mitochondrial protein import pathway is emerging, with conformational alteration a critical feature both preceding and following membrane translocation. The mediators of these steps of conformational alteration, as well as steps of recognition, translocation, and proteolytic cleavage, appear to be proteins. Using powerful tools
A L, Horwich +3 more
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Mitochondrial diseases caused by dysfunctional mitochondrial protein import
Biochemical Society Transactions, 2018Mitochondria are essential organelles which perform complex and varied functions within eukaryotic cells. Maintenance of mitochondrial health and functionality is thus a key cellular priority and relies on the organelle's extensive proteome. The mitochondrial proteome is largely encoded by nuclear genes, and mitochondrial proteins must be sorted to the
Thomas Daniel, Jackson +2 more
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Association of a Novel Mitochondrial Protein M19 with Mitochondrial Nucleoids
The Journal of Biochemistry, 2009We have identified a novel mitochondrial protein, termed M19, by proteomic analysis of mitochondrial membrane proteins from HeLa cells. M19 is highly conserved among vertebrates, and possesses no homologous domains with other known proteins. By northern and western blotting, mouse M19 was shown to be expressed in various tissues, and to be especially ...
Megumi, Sumitani +4 more
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Linking mitochondrial dynamics to mitochondrial protein quality control
Current Opinion in Genetics & Development, 2016Over the last decade, countless discoveries have been made that have expanded our knowledge of mitochondrial biology, and more often than not, these discoveries provided fascinating new insights into the etiology of human disease. For example, advances in mitochondrial genetics exposed the role of mitochondrial mutations in cancer progression, and the ...
Suraiya, Haroon, Marc, Vermulst
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Mitochondrial Proteins Moonlighting in the Nucleus
Trends in Biochemical Sciences, 2015Mitochondria function as cellular energy generators, producing the fuel required to drive biological processes. The response of cells to mitochondrial activity or dysfunction regulates their survival, growth, proliferation, and differentiation. Several proteins that contain mitochondrial-targeting sequences (MTS) also reside in the nucleus and there is
Monaghan, Richard, Whitmarsh, Alan
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Mitochondrial biogenesis: Inhibitors of mitochondrial protein synthesis
Molecular and Cellular Biochemistry, 1977The effects of erythromycin, chloramphenicol, cycloheximide, pyrimethamine, chromate, cadmium, lead, nickel, 4-nitro-quinoline-1-oxide and thioacetamide on yeast and human cells were studied. Inhibition of the synthesis of mitochondrial proteins resulted in the loss of cytochromes as well as in morphological changes in the cellular membranes and ...
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Mitochondrial proteins in neuronal degeneration
Biochemical and Biophysical Research Communications, 2004In this review, we highlight recent findings about the role of some mitochondrial proteins in neurological diseases. Studies in mice gene-deleted for Omi/HtrA2 and AIF showed the involvement of these mitochondrial proteins in selective cell degeneration in the spinal cord and brain.
Dan, Lindholm +2 more
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Association of calcineurin with mitochondrial proteins
Proteins: Structure, Function, and Bioinformatics, 2006AbstractUsing mouse hearts from Swiss Webster mice, calcineurin was immunoprecipitated using commercially available anti‐calcineurin antibody and the resulting complex analyzed by using sodium dodecyl sulfate‐gel electrophoresis with silver staining. Distinct proteins were observed and subjected to in situ trypsin digestion followed by extraction of ...
Abigail M, Tokheim, Bruce L, Martin
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PLANT UNCOUPLING MITOCHONDRIAL PROTEINS
Annual Review of Plant Biology, 2006Uncoupling proteins (UCPs) are membrane proteins that mediate purine nucleotide-sensitive free fatty acid-activated H+ flux through the inner mitochondrial membrane. After the discovery of UCP in higher plants in 1995, it was acknowledged that these proteins are widely distributed in eukaryotic organisms.
Eugenio Vercesi, Anibal +5 more
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