A5060448002- Mitochondrial Function and Pathology
- Metabolism and Genetic Disorders
- Physiological and biochemical adaptations
- ATP Synthase and ATPases Research
- Adipose Tissue and Metabolism
- RNA and protein synthesis mechanisms
- RNA modifications and cancer
- Animal Behavior and Reproduction
- Cancer, Hypoxia, and Metabolism
- Amphibian and Reptile Biology
- Thermal Regulation in Medicine
- Bat Biology and Ecology Studies
- Effects of Environmental Stressors on Livestock
- Pharmacological Receptor Mechanisms and Effects
- Liver physiology and pathology
- Cardiac Ischemia and Reperfusion
- Genetic Neurodegenerative Diseases
- Drug Transport and Resistance Mechanisms
- Metabolomics and Mass Spectrometry Studies
- Neonatal and fetal brain pathology
- Lysosomal Storage Disorders Research
- Amino Acid Enzymes and Metabolism
- Immune cells in cancer
- Sperm and Testicular Function
- Drug-Induced Hepatotoxicity and Protection
Wellcome Centre for Mitochondrial Research
2024
Lund University
2017-2024
Medicon Village
2018-2021
NeuroVive Pharmaceutical (Sweden)
2018-2020
University of Sfax
2009-2016
Laboratory of Molecular Genetics
2013
ABSTRACT Although mitochondrial respiration is believed to explain a substantial part of the variation in resting metabolic rate (RMR), few studies have empirically studied relationship between organismal and cellular metabolism. We therefore investigated RMR permeabilized blood cells wild great tits (Parus major L.). also correlation traits cell count, as normalizing by count method commonly used study In contrast previous studies, our results show that there was no intact (i.e. with...
Statins are the cornerstone of lipid-lowering therapy. Although generally well tolerated, statin-associated muscle symptoms (SAMS) represent main reason for treatment discontinuation. Mitochondrial dysfunction complex I has been implicated in pathophysiology SAMS. The present study proposed to assess concentration-dependent ex vivo effects three statins on mitochondrial respiration viable human platelets and investigate whether a cell-permeable prodrug succinate (complex II substrate) can...
Abstract Many animals downregulate body temperature to save energy when resting (rest‐phase hypothermia). Small birds that winter at high latitudes have comparatively limited capacity for hypothermia and so pay large costs thermoregulation during cold nights. Available evidence suggests this process is fueled by adenosine triphosphate (ATP)‐dependent mechanisms. Most ATP produced oxidative phosphorylation in the mitochondria, but mitochondrial respiration may be lower because of dependence...
There is a growing interest for the possibility of using peripheral blood cells (including platelets) as markers mitochondrial function in less accessible tissues. Only few studies have examined correlation between respiration and muscle tissue, with small sample sizes conflicting results.This study investigated within across Additional analyses were performed to elucidate which cell type would be most useful assessing systemic function.There was significant but weak tissue platelets...
Cytochrome c oxidase is an essential component of the mitochondrial respiratory chain that catalyzes reduction molecular oxygen by reduced cytochrome c. In this study, authors report second mutation associated with Leigh syndrome in blood and buccal mucosa 2 affected members a Tunisian family. It was novel heteroplasmic missense at nucleotide 9478 gene specifying subunit III substituting valine position 91 to alanine highly conserved amino acid. found high mutant load tissues derived from...
Mitochondrial DNA defects were known to be associated with a wide spectrum of human diseases and patients might present range clinical features in various combinations. In the current study, we described patient psychomotor neurodevelopmental delay, mild hyperintensity posterior periventicular white matter, generalized clonic seizures, leukodystrophy, congenital deafness. He also had tetraplegia, central blindness swallowing difficulty. Brain magnetic resonance imaging (MRI) showed...
Metformin is the most common pharmacological treatment for type 2 diabetes. It considered safe but has been associated with development of lactic acidosis under circumstances where plasma concentrations exceed therapeutic levels. Metformin-induced linked to drug's toxic effect on mitochondrial function. Current strategies aim remove drug and correct acidosis. With a mortality 20%, complementary are needed. In this study, it was investigated whether targeting mitochondria agents that bypass...
Acetaminophen is one of the most common over-the-counter pain medications used worldwide and considered safe at therapeutic dose. However, intentional unintentional overdose accounts for up to 70% acute liver failure cases in western world. Extensive research has demonstrated that induction oxidative stress mitochondrial dysfunction are central development acetaminophen-induced injury. Despite insight gained on mechanism acetaminophen toxicity, there still only clinically approved...
Amiodarone is a potent antiarrhythmic drug and displays substantial liver toxicity in humans. It has previously been demonstrated that amiodarone its metabolite (desethylamiodarone, DEA) can inhibit mitochondrial function, particularly complexes I (CI) II (CII) of the electron transport system various animal tissues cell types. The present study, performed human peripheral blood cells, one liver-derived line, primarily aimed at assessing concentration-dependent effects these drugs on...
Pathogenic mitochondrial DNA (mtDNA) mutations leading to dysfunction can cause cardiomyopathy and heart failure. These were described in the mt-tRNA genes protein-coding genes. The aim of this study was identify genetic defect two patients belonging families with cardiac associated a wide spectrum clinical phenotypes. sequencing analysis whole their parents revealed presence known polymorphisms pathogenic extracted from blood leucocytes: heteroplasmic m.3243A > G mutation MT-TL1 gene...
Understanding mitochondrial biology and pathology is key to understanding the evolution of animal form function. However, measurement often involves invasive, or even terminal, sampling, which can be difficult reconcile in wild models longitudinal studies. Non-mammal vertebrates contain mitochondria their red blood cells, exploited for minimally invasive measurement. Several recent bird studies have measured function using isolated cells. Isolation adds time laboratory might associated with...
Cytochrome c oxidase encoded by multiple mitochondrial genes (COXI, COXII, and COXIII) nuclear is an essential component of the respiratory chain that catalyzes reduction molecular oxygen reduced cytochrome c. Subunits COXI COXII are known to play most role in proton pumping electron transfer. In this study we screened somatic gene infertile men suffering from asthenospermia (n=34) comparison normozoospermic (n=32) fertile (n=100) Tunisian population. A novel homoplasmic missense mutation...
Mitochondrial DNA (mtDNA) defects were known to be associated with a large spectrum of human diseases and patients might present wide range clinical features various combinations. Mutations in mitochondrial tRNAs, rRNAs protein-coding genes or large-scale rearrangements have been implicated several cytopathies. myopathies, usually maternally inherited group neuromuscular caused by dysfunction occurring before the age 20 years often begin exercise intolerance, muscle weakness...
Introduction Head and neck squamous cell carcinoma (HNSCC) constitutes a heterogeneous group of cancers. Human papilloma virus (HPV) is associated with subtype HNSCC better response to treatment more favorable prognosis. Mitochondrial function metabolism vary depending on cancer type can be related tumor aggressiveness. This study aims characterize the HPV-positive HPV-negative lines. Methods Oxidative phosphorylation (OXPHOS) glycolysis were assessed in intact cells, four lines using...
Research on birds suggests that extreme weather events during development may have long-lasting consequences form and function. The underlying cellular mechanisms mediating such phenotypic effects are poorly studied. We raised Japanese quail in warm (30oC) or cold (10oC) temperatures from hatching until adulthood, then measured mitochondrial metabolism intact blood cells at representative normothermic body temperature (41oC) a hyperthermic (45oC) commonly attain when heat stressed. To...