A5075650495- Mitochondrial Function and Pathology
- ATP Synthase and ATPases Research
- Genetic Neurodegenerative Diseases
- Metabolism and Genetic Disorders
- Autophagy in Disease and Therapy
- Genetics and Neurodevelopmental Disorders
- Ubiquitin and proteasome pathways
- Synthesis of Organic Compounds
- Ion Channels and Receptors
- Metabolomics and Mass Spectrometry Studies
- Synthesis and biological activity
- Genetic Syndromes and Imprinting
- Video Analysis and Summarization
- Amyotrophic Lateral Sclerosis Research
- Synthesis and Characterization of Pyrroles
- Reproductive biology and impacts on aquatic species
- Epigenetics and DNA Methylation
- Ion channel regulation and function
- Photosynthetic Processes and Mechanisms
- Fish Ecology and Management Studies
- Human Pose and Action Recognition
- MicroRNA in disease regulation
- Human Motion and Animation
- Pluripotent Stem Cells Research
- Physiological and biochemical adaptations
Karolinska Institutet
2023-2025
University of Padua
2016-2025
The mitochondrial contact site and cristae organizing system (MICOS) Optic atrophy 1 (OPA1) control shape, thus affecting function apoptosis. Whether how they physically functionally interact is unclear. Here, we provide evidence that OPA1 epistatic to MICOS in the regulation of shape. Proteomic analysis identifies multiple components native OPA1-containing high molecular weight complexes disrupted during remodeling. MIC60, a core protein, interacts with OPA1, together, junction number...
Abstract Loss-of-function variants in the PRKN gene encoding ubiquitin E3 ligase PARKIN cause autosomal recessive early-onset Parkinson’s disease (PD). Extensive vitro and vivo studies have reported that is involved multiple pathways of mitochondrial quality control, including degradation biogenesis. However, these findings are surrounded by substantial controversy due to conflicting experimental data. In addition, existing PARKIN-deficient mouse models failed faithfully recapitulate PD...
The potassium channel Kv1.3, involved in several important pathologies, is the target of a family psoralen-based drugs whose mechanism action not fully understood. Here we provide evidence for physical interaction mitochondria-located Kv1.3 (mtKv1.3) and Complex I respiratory chain show that this proximity underlies death-inducing ability psoralenic inhibitors. effects PAP-1-MHEG (PAP-1, inhibitor, with six monomeric ethylene glycol units attached to phenyl ring PAP-1), more soluble novel...
Somatic mitochondrial DNA (mtDNA) mutations are heavily implicated as important drivers of ageing and age-related diseases. Their pathological effect can be partially counteracted by increasing the absolute amount wild-type mtDNA via moderately upregulating TFAM, a protein for packaging expression. However, strong TFAM overexpression also have detrimental effects it results in hypercompaction subsequent impairment gene In this study, we experimentally addressed propensity moderate modulation...
Somatic mitochondrial DNA (mtDNA) mutations are heavily implicated as important drivers of ageing and age-related diseases. Their pathological effect can be partially counteracted by increasing the absolute amount wild-type mtDNA via moderately upregulating TFAM, a protein for packaging expression. However, strong TFAM overexpression also have detrimental effects it results in hypercompaction subsequent impairment gene In this study, we experimentally addressed propensity moderate modulation...
Somatic mitochondrial DNA (mtDNA) mutations are heavily implicated as important drivers of ageing and age-related diseases. Their pathological effect can be partially counteracted by increasing the absolute amount wild-type mtDNA via moderately upregulating TFAM, a protein for packaging expression. However, strong TFAM overexpression also have detrimental effects it results in hypercompaction subsequent impairment gene In this study, we experimentally addressed propensity moderate modulation...
Friedreich ataxia (FRDA) is a neurodegenerative disease resulting from severe decrease of frataxin (FXN). Most patients carry GAA repeat expansion in both alleles the FXN gene, whereas small fraction them are compound heterozygous for and point mutation other allele. involved mitochondrial biogenesis FeS-clusters. Distinctive feature FRDA patient cells an impaired cellular respiration, likely due to deficit key redox cofactors working as electrons shuttles through respiratory chain. However,...
Abstract Loss-of-function variants in the PRKN gene encoding ubiquitin E3 ligase PARKIN cause autosomal recessive early-onset Parkinson’s disease (PD). Extensive vitro and vivo studies have reported that is involved multiple pathways of mitochondrial quality control, including degradation biogenesis. However, these findings are surrounded by substantial controversy due to conflicting experimental data. In addition, existing PARKIN-deficient mouse models failed faithfully recapitulate PD...
Somatic mitochondrial DNA (mtDNA) mutations are heavily implicated as important drivers of ageing and age-related diseases. Their pathological effect can be partially counteracted by increasing the absolute amount wild-type mtDNA via moderately upregulating TFAM, a protein for packaging expression. However, strong TFAM overexpression also have detrimental effects it results in hypercompaction subsequent impairment gene In this study, we experimentally addressed propensity moderate modulation...
Sperm fertilisation success depends on both intrinsic quality and the interactions with surrounding reproductive fluids. In several fish species, these have a variable effect sperm performance. Although specific responses to fluids may depend differences in quality, variations traditionally recorded functional traits do not fully account for observed patterns. New methods enhance evaluation of prove be valuable at applied theoretical levels, by improving breeding protocol reared species...