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See detailMitochondrial encephalomyopathy with cytochrome c oxidase deficiency caused by a novel mutation in the MTCO1 gene.
Debray, François-Guillaume ULg; Seneca, Sara; Gonce, Michel et al

in Mitochondrion (2014)

Cytochrome c oxidase (COX) deficiency is one of the most common respiratory chain deficiencies. A woman was presented at the age of 18y with acute loss of consciousness, non-convulsive status epilepticus ... [more ▼]

Cytochrome c oxidase (COX) deficiency is one of the most common respiratory chain deficiencies. A woman was presented at the age of 18y with acute loss of consciousness, non-convulsive status epilepticus, slow neurological deterioration, transient cortical blindness, exercise intolerance, muscle weakness, hearing loss, cataract and cognitive decline. Muscle biopsy revealed ragged-red fibers, COX negative fibers and a significant decreased activity of complex IV in a homogenate. Using next generation massive parallel sequencing of the mtDNA, a novel heteroplasmic mutation was identified in MTCO1, m.7402delC, causing frameshift and a premature termination codon. Single fiber PCR showed co-segregation of high mutant load in COX negative fibers. Mutation in mitochondrially encoded complex IV subunits should be considered in mitochondrial encephalomyopathies and COX negative fibers after the common mtDNA mutations have been excluded. [less ▲]

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See detailMitochondrial function plasticity in Acanthamoeba castellanii during growth in batch culture.
Czarna, M.; Sluse, Francis ULg; jarmuszkiewicz, W.

in Journal of Bioenergetics & Biomembranes (2007), 39

The alterations in mitochondrial bioenergetics during growth in a batch culture of Acanthamoeba castellanii were studied. The capacity of cytochrome pathway-dependent respiration measured in vitro ... [more ▼]

The alterations in mitochondrial bioenergetics during growth in a batch culture of Acanthamoeba castellanii were studied. The capacity of cytochrome pathway-dependent respiration measured in vitro decreased from the intermediary phase, when cell division slowed down. The pattern of the cytochrome pathway capacity changes was paralleled from the intermediary phase by alterations in the amount of total (and reducible) membranous ubiquinone. These changes were accompanied by a decrease in mitochondrial reactive oxygen species production in vitro (when no energy-dissipating system was active), and almost no change in superoxide dismutase activity and protein level, thus indicating an equivalent need for this enzyme in oxidative stress defence in A. castellanii culture. On the other hand, a decrease in the activity and protein level of alternative oxidase and uncoupling protein was observed in vitro, when cells shifted from the exponential growth phase to the stationary phase. It turned out that the contribution of both energy-dissipating systems in the prevention of mitochondrial reactive oxygen species generation in vivo could lead to its constant level throughout the growth cycle of A. castellanii batch culture. Hence, the observed functional plasticity insures survival of high quality cysts of A. castellanii cells. [less ▲]

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See detailMitochondrial genetics
Remacle, Claire ULg; Matagne, René-Fernand ULg

in Rochaix, Jean-David; Goldschmidt-Clermont, Michel; Merchant, Sabeeha (Eds.) The Molecular Biology of Chloroplasts and Mitochondria in Chlamydomonas (1998)

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See detailMitochondrial Genome Transmission in Chlamydomonas Diploids Obtained by Sexual Crosses and Artificial Fusions: Role of the Mating Type and of a 1 Kb Intron
Remacle, Claire ULg; Bovie, C.; Michel-Wolwertz, M. R. et al

in Molecular & General Genetics [=MGG] (1990), 223(2), 180-4

The linear mitochondrial DNAs of the two infertile algal species Chlamydomonas smithii and C. reinhardtii are co-linear with the exception of a 1 kb intron (alpha intron) located in the cytochrome b gene ... [more ▼]

The linear mitochondrial DNAs of the two infertile algal species Chlamydomonas smithii and C. reinhardtii are co-linear with the exception of a 1 kb intron (alpha intron) located in the cytochrome b gene of C. smithii. C. smithii also possesses an additional HpaI restriction site (H marker) located in the COXI gene, about 5 kb from the intron. In reciprocal crosses, C. smithii (H+ alpha +) x C. reinhardtii (H- alpha -), the alpha intron is transmitted to all diploid progeny, whereas the H marker is frequently transmitted either biparentally or paternally depending on whether the C. smithii parent is maternal (mt+) or paternal (mt-). In diploids resulting from artificial fusion between vegetative cells, the absolute transmission of alpha is accompanied by the frequent transmission of the H+ marker, irrespective of the mating type of the parental strains. Finally, in reciprocal crosses between C. smithii (H+ alpha +) and recombinant H- alpha + clones, the transmission of the H marker is predominantly paternal or biparental. These results allow us to conclude that (1) the alpha intron behaves as a group I intron whose unidirectional conversion influences the transmission of the H marker; and (2) the mt- paternal mitochondrial genome is transmitted more often than the mt+. The mating type has no effect in diploids obtained by artificial fusion. [less ▲]

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See detailThe mitochondrial genome
Cardol, Pierre ULg; Remacle, Claire ULg

in Stern, David; Harris, Elizabeth; Witman, George (Eds.) The Chlamydomonas Sourcebook 3-vol set, 1-3 (2009)

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See detailA mitochondrial half-size ABC transporter is involved in cadmium tolerance in Chlamydomonas reinhardtii
Hanikenne, Marc ULg; Motte, Patrick ULg; Wu, Madeline C.S. et al

in Plant Cell and Environment (2005), 28(7), 863-873

Five cadmium-sensitive insertional mutants, all affected at the CDS1 ('cadmium-sensitive 1') locus, have been previously isolated in the unicellular green alga Chlamydomonas reinhardtii. We here describe ... [more ▼]

Five cadmium-sensitive insertional mutants, all affected at the CDS1 ('cadmium-sensitive 1') locus, have been previously isolated in the unicellular green alga Chlamydomonas reinhardtii. We here describe the cloning of the Cds1 gene (8314 bp with 26 introns) and the corresponding cDNA. The Cds1 gene, strongly induced by cadmium, encodes a putative protein (CrCds1) of 1062 amino acid residues that belongs to the ATM/HMT subfamily of half-size ABC transporters. This subfamily includes both vacuolar HMT-type proteins transporting phytochelatin-cadmium complexes from the cytoplasm to the vacuole and mitochondrial ATM-type proteins involved in the maturation of cytosolic Fe/S proteins. Unlike the Delta sphmt1 cadmium-sensitive mutant of Schizosaccharomyces pombe that lacks a vacuolar HMT-type transporter, the cds1 mutant accumulates a high amount of phytochelatin-cadmium complexes. By epitope tagging, the CrCds1 protein was localized in the mitochondria. Even though mitochondria of cds1 do not accumulate important amounts of 'free' iron, the mutant cells are hypersensitive to high iron concentrations. Our data show for the first time that a mitochondrial ATM-like transporter plays a major role in tolerance to cadmium. [less ▲]

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See detailMitochondrial metabolite carrier family, topology, structure and functional properties: an overview.
Sluse, Francis ULg

in Acta Biochimica Polonica. Polish. (1996), 43(2), 349-360

A set of metabolite carriers operates the traffic of numerous molecules consumed or produced in mitochondrial matrix and/or cytosolic compartments. As their existence has been predicted by the ... [more ▼]

A set of metabolite carriers operates the traffic of numerous molecules consumed or produced in mitochondrial matrix and/or cytosolic compartments. As their existence has been predicted by the chemiosmotic theory, the first challenge, in the late sixties, was to prove their presence in the inner mitochondrial membrane and to describe the various transports carried out. The second challenge was to understand their mechanisms by the kinetic approach in intact mitochondria (seventies). The third challenge (late seventies-eighties) was to isolate and to reconstitute the carriers in liposomes in order to characterize the proteins and to establish the concept of a structural and a functional family as well as some structure-function relationship with the help of primary sequences. Genetics, molecular biology and genomic sequencing bring the fourth challenge (nineties): a raising number of putative carriers becomes known only by their primary sequences but their functions have to be discovered. The actual challenge of the future is the elucidation of the ternary structure of carrier proteins that together with site-directed mutagenesis and kinetic mechanism will permit to advance in the understanding of molecular mechanisms of transport processes. [less ▲]

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See detailMitochondrial NADH:ubiquinone oxidoreductase (complex I) in eukaryotes: A highly conserved subunit composition highlighted by mining of protein databases
Cardol, Pierre ULg

in Biochimica et Biophysica Acta-Bioenergetics (2011), 11

Complex I (NADH:ubiquinone oxidoreductase) is the largest enzyme of the mitochondrial respiratory chain. Compared to its bacterial counterpart which encompasses 14-17 subunits, mitochondrial complex I has ... [more ▼]

Complex I (NADH:ubiquinone oxidoreductase) is the largest enzyme of the mitochondrial respiratory chain. Compared to its bacterial counterpart which encompasses 14-17 subunits, mitochondrial complex I has almost tripled its subunit composition during evolution of eukaryotes, by recruitment of so-called accessory subunits, part of them being specific to distinct evolutionary lineages. The increasing availability of numerous broadly sampled eukaryotic genomes now enables the reconstruction of the evolutionary history of this large protein complex. Here, a combination of profile-based sequence comparisons and basic structural properties analyses at the protein level enabled to pinpoint homology relationships between complex I subunits from fungi, mammals or green plants, previously identified as "lineage-specific" subunits. In addition, homologs of at least 40 mammalian complex I subunits are present in representatives of all major eukaryote assemblages, half of them having not been investigated so far (Excavates, Chromalveolates, Amoebozoa). This analysis revealed that complex I was subject to a phenomenal increase in size that predated the diversification of extant eukaryotes, followed by very few lineage-specific additions/losses of subunits. The implications of this subunit conservation for studies of complex I are discussed. [less ▲]

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See detailMitochondrial oxidative phosphorylation : in vitro and in situ effect of EGb 761
Willet, K.; DETRY, Olivier ULg; Evens, A. et al

in Packer, L.; Trabet, Maret G; Xin, Wenjuan (Eds.) Proceedings of the international symposium on natural antioxidants molecular mechanisms and health effects (1996)

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See detailMitochondrial oxidative phosphorylation injuries occurring in situ and in vitro.
Willet, K.; Vaz de Macedo, D.; DETRY, Olivier ULg et al

in Transplantation Proceedings (1995), 27

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See detailThe mitochondrial oxidative phosphorylation proteome of Chlamydomonas reinhardtii deduced from the genome sequencing project
Cardol, Pierre ULg; Gonzalez-Halphen, Diego; Reyes-Prieto, Adrian et al

in Plant Physiology (2005), 137(2), 447-459

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See detailMitochondrial phylogeography of the edible dormouse (Glis glis) in the western Palearctic region
Hurner, Helene; Krystufek, Boris; Sara, Maurizio et al

in Journal of Mammalogy (2010), 91(1), 233-242

This study describes in detail the phylogeoraphic pattern Of the edible dormouse (Glis glis) a European rodent With pronounced hibernating behavior We Used sequences of 831 base pairs of the mitochondrial ... [more ▼]

This study describes in detail the phylogeoraphic pattern Of the edible dormouse (Glis glis) a European rodent With pronounced hibernating behavior We Used sequences of 831 base pairs of the mitochondrial DNA cytochrome-b gene from 130 edible dormice collected at 43 localities (throughout Its distribution. Our results reveal presence of 3 main haplogroups: Sicilian, South Italian (restricted to the Calabrian region) (a widespread lineage corresponding to all remaining western, central. and eastern European populations). Examination of paleontological data confirms refugial regions for G,Its in the 3 Mediterranean peninsulas, although overall low genetic diversity is found. The low diversity of the European lineage Is probably the result refugium. Other factors, such as the of a recent expansion (dated around 2.000( years ago) from a single ecological constraints oil the species, way have caused genetic bottlenecks that reinforced the low genetic variability of G glis. This work could have important implications for strategies to conserve the edible dormouse by defining important areas for their conservation DOI: 10.1644/08-MAMM-A-392R1.1 [less ▲]

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See detailMitochondrial phylogeography of the Woodmouse (Apodemus sylvaticus) in the Western Palearctic region.
Michaux, Johan ULg; Magnanou, E.; Paradis, E. et al

in Molecular Ecology (2003), 12(3), 685-97

We sequenced 965 base pairs of the mitochondrial DNA cytochrome b from 102 woodmice (Apodemus sylvaticus) collected from 40 European localities. The aims of the study were to answer the following ... [more ▼]

We sequenced 965 base pairs of the mitochondrial DNA cytochrome b from 102 woodmice (Apodemus sylvaticus) collected from 40 European localities. The aims of the study were to answer the following questions. (i) Did the Mediterranean peninsulas play a role as refuge for woodmice? (ii) Is genetic variability of A. sylvaticus higher in the Mediterranean region compared with northern Europe? (iii) Are the patterns of the postglacial colonization of Europe by woodmice similar to those presently recognized for other European species? The results provide a clear picture of the impact of the Quaternary glaciations on the genetic and geographical structure of the woodmouse. Our analyses indicate a higher genetic variability of woodmice in the Mediterranean peninsulas compared to northern Europe, suggesting a role of the former as refuge regions for this small mammal. An original pattern of postglacial colonization is proposed where the Iberian and southern France refuge populations colonized almost all European regions. The Sicilian population appears to be very differentiated and highly variable. This emphasizes the importance of this island as a 'hot spot' for the intraspecific genetic diversity of the woodmouse. Finally, woodmice in North Africa originated from southwestern Europe, most probably as a result of a recent anthropogenic introduction. [less ▲]

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See detailMitochondrial Physiology Network: Mitochondrial respiration in permeabilized muscle fibers: needle biopsy from horse skeletal muscle
Lemieux, Hélène; Votion, Dominique ULg; Gnaiger, Erich

in Gnaiger, Erich (Ed.) MipNet Publications (2007)

1 Introduction ..........................................................1 2 The Protocol: Respiratory States .............................2 2.1 The O2k demo experiment ... [more ▼]

1 Introduction ..........................................................1 2 The Protocol: Respiratory States .............................2 2.1 The O2k demo experiment ...................................2 2.2 Preparation of permeabilized fibres .......................3 2.3 The experimental protocol ....................................3 3 References ............................................................4 [less ▲]

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See detailMitochondrial Proteomics of a Secondary Green Alga
Perez, Emilie ULg; Degand, Hervé; Morsomme, Pierre et al

Poster (2012, June)

Euglena gracilis is an alga that derives from a secondary endosymbiosis with a green alga. Our general objective is to study the interactions established between the chloroplast and the mitochondrion ... [more ▼]

Euglena gracilis is an alga that derives from a secondary endosymbiosis with a green alga. Our general objective is to study the interactions established between the chloroplast and the mitochondrion during the endosymbiosic event and to determine the phylogenetic origin of the genes encoding the proteins involved in these interactions. As a first step, we performed a high-throughput analysis of the mitochondrial proteome of Euglena gracilis. Our MS/MS experiments mostly recover mitochondrial proteins representing 15 mitochondrial pathways, which indicates that our mitochondrial extracts are relatively pure, but the phylogenetic origins of the corresponding genes are surprisingly diverse. [less ▲]

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See detailMitochondrial respiratory capacity in skeletal muscle from top-level equine athletes
Votion, Dominique ULg

in FEBS Workshop: the many functions of the organism in our cells (2010, October 27)

We have recently introduced the muscle microbiopsy technique to study oxidative phosphorylation (OXPHOS) capacity in horses by high-resolution respirometry (HRR). Mitochondrial respiration is measured in ... [more ▼]

We have recently introduced the muscle microbiopsy technique to study oxidative phosphorylation (OXPHOS) capacity in horses by high-resolution respirometry (HRR). Mitochondrial respiration is measured in the course of specially designed multiple substrate-uncoupler-inhibitor titration (SUIT) protocols. This new technique is proposed for routine monitoring of horses in training as an aid to improve training program and hopefully to reduce exercise-induced muscle injury. [less ▲]

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See detailMitochondrial respiratory chain complex patterns from Acanthamoeba castellanii and Lycopersicon esculentum: comparative analysis by BN-PAGE and evidence of protein-protein interaction between alternative oxidase and complex III.
Navet, R.; Jarmuszkiewicz, W.; Douette, P. et al

in Journal of Bioenergetics & Biomembranes (2004), 36(5), 471-479

We have previously shown that a kinetic interplay exists between the cytochrome pathway and the alternative oxidase in mitochondria from amoeba Acanthamoeba castellanii . Native interaction analyses using ... [more ▼]

We have previously shown that a kinetic interplay exists between the cytochrome pathway and the alternative oxidase in mitochondria from amoeba Acanthamoeba castellanii . Native interaction analyses using blue native gel electrophoresis coupled to denaturating electrophoresis and immunodetection have indicated associations between alternative oxidase and oxidative phosphorylation complexes in both amoeba and tomato mitochondria. These associations are dependent on the expression level of alternative oxidase according to the physiological state in both organisms. Alternative oxidase associates broadly with large complexes of the respiratory chain when it is expressed in large amount, i.e., in ripe tomato and exponentially growing amoeba. On the contrary, alternative oxidase interacts specifically with complex III even if expression of the oxidase is low, i.e., in green tomato and stationary phase amoeba. This specific interaction represents a higher level of regulation driven by protein-protein interactions leading to a direct kinetic interplay between the cytochrome pathway and alternative oxidase in both plant and amoeba mitochondria. [less ▲]

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