Browsing
     by title


0-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z

or enter first few letters:   
OK
Full Text
Peer Reviewed
See detailThe mitochondria-related phenotype in the osteoarthritis disease
Rego-Perez, I; Fernadez-Moreno, M; DEBERG, Michelle ULg et al

in Osteoarthritis and Cartilage (2010)

Detailed reference viewed: 14 (0 ULg)
Peer Reviewed
See detailMitochondrial and cytoplasmic asparaginyl-tRNA synthetases from S. cerevisiae.
Landrieu, Isabelle; Vandenbol, Micheline ULg; Hartlein, M. et al

Poster (1997)

Detailed reference viewed: 7 (0 ULg)
Peer Reviewed
See detailMitochondrial Asparaginyl-Trna Synthetase Is Encoded By The Yeast Nuclear Gene Ycr24c
Landrieu, I.; Vandenbol, Micheline ULg; Hartlein, M. et al

in European Journal of Biochemistry (1997), 243(1-2),

Detailed reference viewed: 6 (0 ULg)
Full Text
Peer Reviewed
See detailThe mitochondrial ATP synthase of chlorophycean algae contains eight subunits of unknown origin involved in the formation of an atypical stator-stalk and in the dimerization of the complex
Vazquez-Acevedo, Miriam; Cardol, Pierre ULg; Cano-Estrada, Araceli et al

in Journal of Bioenergetics & Biomembranes (2006), 38(5-6), 271-282

Mitochondrial F1FO-ATP synthase of Chlamydomonas reinhardtii and Polytomella sp. is a dimer of 1,600,000 Da. In Chlamydomonas the enzyme lacks the classical subunits that constitute the peripheral stator ... [more ▼]

Mitochondrial F1FO-ATP synthase of Chlamydomonas reinhardtii and Polytomella sp. is a dimer of 1,600,000 Da. In Chlamydomonas the enzyme lacks the classical subunits that constitute the peripheral stator-stalk as well as those involved in the dimerization of the fungal and mammal complex. Instead, it contains eight novel polypeptides named ASA1 to 8. We show that homologs of these subunits are also present in the chlorophycean algae Polytomella sp. and Volvox carterii. Blue Native Gel Electrophoresis analysis of mitochondria from different green algal species also indicates that stable dimeric mitochondrial ATP synthases may be characteristic of all Chlorophyceae. One additional subunit, ASA9, was identified in the purified mitochondrial ATP synthase of Polytomella sp. The dissociation profile of the Polytomella enzyme at high-temperatures and cross-linking experiments finally suggest that some of the ASA polypeptides constitute a stator-stalk with a unique architecture, while others may be involved in the formation of a highly-stable dimeric complex. The algal enzyme seems to have modified the structural features of its surrounding scaffold, while conserving almost intact the structure of its catalytic subunits. [less ▲]

Detailed reference viewed: 73 (3 ULg)
Full Text
Peer Reviewed
See detailMitochondrial comparative proteomics: Strenghts and Pitfalls
Mathy, Grégory ULg; Sluse, Francis ULg

in Biochimica et Biophysica Acta-Bioenergetics (2008), 1977

In this review, we describe the various techniques available to carry out valid comparative proteomics, their advantages and their disadvantages according to the goal of the research. Two-dimensional ... [more ▼]

In this review, we describe the various techniques available to carry out valid comparative proteomics, their advantages and their disadvantages according to the goal of the research. Two-dimensional electrophoresis and 2D-DIGE are compared to shotgun proteomics and SILE. We give our opinion on the best fields of application in the domain of comparative proteomics. We emphasize the usefulness of these new tools, providing mass data to study physiology and mitochondrial plasticity when faced with a specific mitochondrial insufficiency or exogenic stress. We illustrate the subject with results obtained in our laboratory specifying the importance of an approach of comparative proteomics combined from mitochondria and from the cell, which makes it possible to obtain important information on the status of the mitochondrial function at the cellular level. Finally, we draw attention to the dangers of the extrapolation of proteomic data to metabolic flows which requires the greatest care [less ▲]

Detailed reference viewed: 37 (13 ULg)
Full Text
Peer Reviewed
See detailMitochondrial complex I encephalomyopathy and cerebral 5-methyltetrahydrofolate deficiency
RAMAEKERS, Vincent ULg; Wels, J.; Sequeira, J. M. et al

in Neuropediatrics (2007), 38(4), 184-187

Folate transport to the brain depends on ATP-driven folate receptor-mediated transport across choroid plexus epithelial cells. Failure of ATP production in Kearns-Sayre syndrome syndrome provides one ... [more ▼]

Folate transport to the brain depends on ATP-driven folate receptor-mediated transport across choroid plexus epithelial cells. Failure of ATP production in Kearns-Sayre syndrome syndrome provides one explanation for the finding of low spinal fluid (CSF) 5-methyltetrahydrofolate (5MTHF) levels in this condition. Therefore, we suspect the presence of reduced folate transport across the blood-spinal fluid barrier in other mitochondrial encephalopathies. In the present patient with mitochondrial complex I encephalomyopathy a low 5-methyltetrahydrofolate level was found in the CSF. Serum folate receptor autoantibodies were negative and could not explain the low spinal fluid folate levels. The epileptic seizures did not respond to primidone monotherapy, but addition of ubiquinone-10 and radical scavengers reduced seizure frequency. Add-on treatment with folinic acid led to partial clinical improvement including full control of epilepsy, followed by marked recovery from demyelination of the brainstem, thalamus, basal ganglia and white matter. Cerebral folate deficiency is not only present in Kearns-Sayre syndrome but may also be secondary to the failure of mitochondrial ATP production in other mitochondrial encephalopathies. Treatment with folinic acid in addition to supplementation with radical scavengers and cofactors of deficient respiratory enzymes can result in partial clinical improvement and reversal of abnormal myelination patterns on neuro-imaging. © Georg Thieme Verlag KG Stuttgart. [less ▲]

Detailed reference viewed: 46 (1 ULg)
Full Text
Peer Reviewed
See detailMitochondrial diseases associated with cerebral folate deficiency
Garcia-Cazorla, A.; Quadros, E. V.; Nascimento, A. et al

in Neurology (2008), 70(16), 1360-1362

Detailed reference viewed: 16 (1 ULg)
Full Text
Peer Reviewed
See detailMitochondrial DNA haplogroups and serum levels of proteolytic enzymes in patients with osteoarthritis.
Rego-Perez, I.; Fernandez-Moreno, M.; Deberg, Michelle ULg et al

in Annals of the Rheumatic Diseases (2011), 70(4), 646-52

OBJECTIVE: To analyse the influence of mitochondrial DNA haplogroups, as well as the radiographic grade, on serum levels of proteolytic enzymes in patients with osteoarthritis (OA). METHODS: Serum levels ... [more ▼]

OBJECTIVE: To analyse the influence of mitochondrial DNA haplogroups, as well as the radiographic grade, on serum levels of proteolytic enzymes in patients with osteoarthritis (OA). METHODS: Serum levels of metalloproteinase-1 (MMP-1), MMP-3, MMP-13, myeloperoxidase and cathepsin K were analysed in 73 patients with OA and 77 healthy controls carrying the haplogroups J, U and H, by ELISA. Knee and hip radiographs were classified according to Kellgren and Lawrence (K/L) scoring from grade 0 to grade IV. Non-parametric and multiple regression analyses were performed to test the effects of clinical variables, including gender, age, smoking status, diagnosis, haplogroups and radiological K/L grade on serum levels of these enzymes. RESULTS: A significant influence of the haplogroups on the serum levels of MMP-3 and MMP-13 was detected (p=0.027 and p=0.035, respectively). Patients with OA with haplogroup H showed higher serum levels of MMP-3 than healthy controls. Serum levels of MMP-13 were significantly higher in patients with OA (p<0.001), and carriers of the haplogroup J showed lower levels than H carriers. Besides, levels of MMP-13 were proportionally higher in radiological groups B (K/L grade II and III) and C (K/L grade IV) than in group A (K/L grade 0 and I) (p=0.005). CONCLUSIONS: This study shows that haplogroups have a significant influence on serum levels of MMP-3 and MMP-13. The influence of the haplogroups on serum levels of MMP-3 is clearly dependent on the diagnosis, whereas the influence of the haplogroups on serum levels of MMP-13 is independent of diagnosis. [less ▲]

Detailed reference viewed: 40 (4 ULg)
Full Text
Peer Reviewed
See detailMitochondrial DNA haplogroups influence response to Riboflavin in Migraineurs
Di Lorenzo, C.; Coppola, G.; Santorelli, F. et al

in Cephalalgia : An International Journal of Headache (2009, January), 29(1),

Detailed reference viewed: 33 (5 ULg)
Full Text
Peer Reviewed
See detailMitochondrial DNA haplogroups influence the therapeutic response to riboflavin in migraineurs.
Di Lorenzo, C.; Pierelli, F.; Coppola, G. et al

in Neurology (2009), 72(18), 1588-94

OBJECTIVES: In migraine, an interictal reduction of mitochondrial energy metabolism and a preventive effect of high-dose riboflavin were reported. To explore the relation between the two, we tested if the ... [more ▼]

OBJECTIVES: In migraine, an interictal reduction of mitochondrial energy metabolism and a preventive effect of high-dose riboflavin were reported. To explore the relation between the two, we tested if the therapeutic response to riboflavin is associated with specific mitochondrial DNA (mtDNA) haplogroups. We focused our attention on haplogroup H, which is known to differ from others in terms of energy metabolism. METHODS: Sixty-four migraineurs completed a 4-month open trial with riboflavin (400 mg QD) and were genotyped blindly for mtDNA haplogroups. RESULTS: Forty patients responded to riboflavin treatment and 24 were nonresponders. The mtDNA haplogroup H was found in 29 subjects (20 migraine without aura, 9 migraine with aura). Riboflavin responders were more numerous in the non-H group (67.5%). Conversely, nonresponders were mostly H (66.7%). The difference between the two groups was significant (chi(2) = 7.07; p = 0.01). The presence of aura had no influence on riboflavin's effectiveness (chi(2) = 0.113; p = 0.74) and was not associated with a particular haplogroup (chi(2) = 0.55; p = 0.46). CONCLUSIONS: In this pharmacogenetic study, riboflavin appears to be more effective in patients with migraine with non-H mitochondrial DNA haplotypes. The underlying mechanisms are unknown, but could be related to the association of haplogroup H with increased activity in complex I, which is a major target for riboflavin. Our results may have ethnic implications, since haplogroup H is chiefly found in the European population. [less ▲]

Detailed reference viewed: 54 (3 ULg)
Full Text
Peer Reviewed
See detailMitochondrial DNA haplogroups modulate the serum levels of biomarkers in patients with osteoarthritis
Rego, I; Fernandez, M; DEBERG, Michelle ULg et al

in Osteoarthritis and Cartilage (2009), 17(Suppl 1), 72

Detailed reference viewed: 3 (0 ULg)
Full Text
Peer Reviewed
See detailMitochondrial DNA haplogroups modulate the serum levels of biomarkers in patients with osteoarthritis.
Rego-Perez, I.; Fernandez-Moreno, M.; Deberg, Michelle ULg et al

in Annals of the Rheumatic Diseases (2010), 69(5), 910-7

OBJECTIVE: To analyse the influence of mitochondrial DNA (mtDNA) haplogroups on serum levels of molecular biomarkers in patients with osteoarthritis (OA). METHODS: Serum levels of molecular biomarkers of ... [more ▼]

OBJECTIVE: To analyse the influence of mitochondrial DNA (mtDNA) haplogroups on serum levels of molecular biomarkers in patients with osteoarthritis (OA). METHODS: Serum levels of molecular biomarkers of cartilage metabolism (collagen type II markers: C-terminal neoepitope generated by the collagenase-mediated cleavage of collagen type II triple helix (C2C), collagen type II (Coll2-1, and its nitrated form, Coll2-1NO(2)), procollagen type II (CPII)), synovial metabolism (hyaluronic acid (HA)) and cartilage and synovial turnover (cartilage glycoprotein 39 (YKL-40)) were analysed in 73 patients with OA and 77 healthy controls using ELISAs. All participants had been previously genotyped for the mtDNA haplogroups J, U and H. Non-parametric and multivariate analysis were performed to test the effects of the clinical variables, including gender, age, smoking status, diagnosis, mtDNA haplogroups and radiological Kellgren and Lawrence (K/L) grade on the serum levels of the molecular markers. RESULTS: Non-parametric analysis found increased serum levels of HA in patients with OA, while the values for C2C and the C2C/CPII ratio were significantly higher in the healthy controls. A multiple regression analysis showed a relationship between the mtDNA haplogroups and serum levels of the typical collagen type II markers. Carriers of the mtDNA haplogroup H had higher levels while carriers of the mtDNA haplogroup J showed lower levels. Statistically significant interactions between mtDNA haplogroups and diagnosis and between mtDNA haplogroups and radiological K/L grade in the serum levels of molecular markers were also found. CONCLUSION: A new role for mtDNA haplogroups emerges from this work. The results suggest that the mtDNA haplogroups interact significantly with the serum levels of OA-related molecular markers, suggesting the possibility of their use as a complementary assay with these molecular markers. [less ▲]

Detailed reference viewed: 58 (0 ULg)
See detailMitochondrial dysfunction in HDAC5-depleted cancer cells induces glucose-dependent metabolic adaptation
Hendrick, Elodie ULg; Matheus, Nicolas ULg; Peixoto, Paul ULg et al

Poster (2013, May 17)

Introduction: Histone deacetylases (HDAC) is a family of eighteen enzymes, which modulates the acetylation level of histones and non-histone proteins to regulate gene expression and chromatin structure ... [more ▼]

Introduction: Histone deacetylases (HDAC) is a family of eighteen enzymes, which modulates the acetylation level of histones and non-histone proteins to regulate gene expression and chromatin structure. Broad-spectrum inhibitors of these enzymes such as SAHA can inhibit tumor growth both in vitro and in vivo and are currently used as anti-cancer agents in clinic. For many years, we are investigating the specific role of individual HDAC members in cancer biology and we have recently demonstrated that depletion of HDAC5 using siRNA technology triggered cancer cells to both autophagy and apoptosis1. Aims: The goal of this study is to further investigate the molecular mechanisms by which HDAC5 depletion induces both autophagy and apoptosis in cancer cells. Results: Screening transcriptomic study demonstrated that HDAC5 depletion induces a deregulation of genes encoding subunits of complex I of the mitochondrial respiratory chain leading to a significant increase of ROS production. This ROS accumulation promotes autophagy including mitophagy. Indeed, pretreatment with NAC, a ROS scavenger, blocked autophagy triggered by HDAC5 silencing. This autophagy seems to be protective as its blocking with NAC, chloroquine or bafilomycin A1 enhances pro-apoptotic effect of HDAC5 depletion. In addition, mitochondrial dysfunction provokes metabolism adaptation associated with increase of the importance of glucose metabolism in HDAC5 depleted cancer cells. Indeed, low-glucose culture of HDAC5-depleted cells significantly increases apoptotic cell death suggesting that glucose deprivation might be combined to HDAC5 inhibition as a therapeutic strategy to kill cancer cells. Conclusion: Our study demonstrated for the first time that specific HDAC5 inhibition induces alteration of gene expression encoding mitochondrial proteins in cancer cells and provide insight into a valuable experimental strategy for manipulation of specific HDAC5 inhibition and glucose metabolism in therapy against cancer. 1.Peixoto, P. et al. HDAC5 is required for maintenance of pericentric heterochromatin, and controls cell-cycle progression and survival of human cancer cells. Cell death and differentiation, 2012; 1-14. [less ▲]

Detailed reference viewed: 24 (2 ULg)
Full Text
Peer Reviewed
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 ▲]

Detailed reference viewed: 46 (1 ULg)
Full Text
Peer Reviewed
See detailMitochondrial function and aerobic capacity assessed by high resolution respirometry in Thoroughbred horses
Serteyn, Didier ULg; Ceusters, Justine ULg; Nonnenmacher et al

in Comparative Exercise Physiology (2016), 12(2), 67-73

During the initial stages of training of young Thoroughbred horses, low intensity exercise is employed to increase aerobic capacity. High Resolution Respirometry (HRR) allows the determination of aerobic ... [more ▼]

During the initial stages of training of young Thoroughbred horses, low intensity exercise is employed to increase aerobic capacity. High Resolution Respirometry (HRR) allows the determination of aerobic capacities in small samples of permeabilised muscle fibres. The aim of the study was to measure the mitochondrial function by HRR in Thoroughbred horses, to compare these values to Warmblood horses and to evaluate the effect of a 10-weeks training period. The mitochondrial function was measured by HRR using different substrate-uncoupler protocols (SUIT 1 and 2) in muscle microbiopsies from two groups of untrained horses: 17 Warmblood and 8 Thoroughbred and in the group of 8 Thoroughbred horses before and after a 10-week training period. The SUIT1 protocol employed to compare the two groups of horses showed that in Thoroughbred horses, the mean values for oxygen flux expressed as tissue mass-specific respiration were significantly higher for complex I (CI)Glutamate+Malate, CI + complex II, and maximum electron transport capacities (ETSmax) than the mean values measured in Warmblood horses. The SUIT 1 and SUIT 2 protocols revealed large differences among Thoroughbred horses before and after training. The SUIT 2 protocols showed a significant difference for the complex I activity before and after training but only when the oxygen flux was expressed as percentage of ETSmax. This study shows the interest of HRR in equine sport medicine and exercise physiology, but shows that the technique requires further refinement. Indeed significant differences have been shown between the Thoroughbred and the Warmblood horses highlighting the need to have baseline data for each breed. The Thoroughbred horses had globally a high oxidative phosphorylation capacity with an increase of CI activity induced by an aerobic training program. [less ▲]

Detailed reference viewed: 3 (0 ULg)
Full Text
Peer Reviewed
See detailMitochondrial function is altered in horse atypical myopathy
Lemieux, Hélène; BOEMER, François ULg; van Galen, Gaby et al

in Mitochondrion (2016), 30

Equine atypical myopathy in Europe is a fatal rhabdomyolysis syndrome that results from the ingestion of hypoglycin A contained in seeds and seedlings of Acer pseudoplatanus. The hallmark of atypical ... [more ▼]

Equine atypical myopathy in Europe is a fatal rhabdomyolysis syndrome that results from the ingestion of hypoglycin A contained in seeds and seedlings of Acer pseudoplatanus. The hallmark of atypical myopathy consists of a severe alteration in the energy metabolism including a severe impairment in muscle mitochondrial respiration that could contribute to its high death rate [less ▲]

Detailed reference viewed: 23 (7 ULg)
Full Text
Peer Reviewed
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 ▲]

Detailed reference viewed: 14 (2 ULg)