A5030414824- Mitochondrial Function and Pathology
- Genetics and Neurodevelopmental Disorders
- Endoplasmic Reticulum Stress and Disease
- Autophagy in Disease and Therapy
- Adipose Tissue and Metabolism
- Pancreatic function and diabetes
- Peroxisome Proliferator-Activated Receptors
- Parkinson's Disease Mechanisms and Treatments
- Metabolism and Genetic Disorders
- RNA and protein synthesis mechanisms
- ATP Synthase and ATPases Research
- Genomics and Rare Diseases
- Renin-Angiotensin System Studies
- Advanced Glycation End Products research
- Nuclear Receptors and Signaling
- Diet and metabolism studies
- Glycogen Storage Diseases and Myoclonus
- Sirtuins and Resveratrol in Medicine
- Mechanisms of cancer metastasis
University of Tartu
2013-2024
Center for Translational Molecular Medicine
2014
Mitochondrial fusion-fission dynamics play a crucial role in many important cell processes. These control mitochondrial morphology, which turn influences several properties including bioenergetics and quality control, they appear to be affected neurodegenerative diseases. However, an integrated quantitative understanding of how morphology has not yet been described. Here, we took advantage modern visualisation techniques provide clear explanation fusion fission correlate with length motility...
Deficiency of the protein Wolfram syndrome 1 (WFS1) is associated with multiple neurological and psychiatric abnormalities similar to those observed in pathologies showing alterations mitochondrial dynamics. The aim this study was examine hypothesis that WFS1 deficiency affects neuronal function via abnormalities. We show down-regulation neurons leads dramatic changes dynamics (inhibited fusion, altered trafficking, augmented mitophagy), delaying development. induces endoplasmic reticulum...
The autophagy protein BECN1/Beclin 1 is known to play a central role in autophagosome formation and maturation. results presented here demonstrate that BECN1 interacts with the Parkinson disease-related PARK2. This interaction does not require PARK2 translocation mitochondria occurs mostly cytosol. However, our suggest involved because loss of inhibits CCCP- or PINK1 overexpression-induced translocation. Our also observed PARK2-BECN1 functionally important. Measurements level MFN2 (mitofusin...
Article30 November 2018Open Access Source DataTransparent process Miro proteins prime mitochondria for Parkin translocation and mitophagy Dzhamilja Safiulina Corresponding Author [email protected] orcid.org/0000-0002-4188-4207 Department of Pharmacology, Institute Biomedicine Translational Medicine, University Tartu, Estonia Search more papers by this author Malle Kuum orcid.org/0000-0002-4681-1859 Vinay Choubey Nana Gogichaishvili Joanna Liiv Miriam A Hickey Michal Cagalinec Merle Mandel...
When ROS production exceeds the cellular antioxidant capacity, cell needs to eliminate defective mitochondria responsible for excessive production. It has been proposed that removal of these involves mitophagy, but mechanism this regulation remains unclear. Here, we demonstrate moderate mitochondrial superoxide and hydrogen peroxide oxidates KEAP1, thus breaking interaction between protein PGAM5, leading inhibition its proteasomal degradation. Accumulated PGAM5 interferes with processing...
During early development, neurons undergo complex morphological rearrangements to assemble into neuronal circuits and propagate signals. Rapid growth requires a large quantity of building materials, efficient intracellular transport also considerable amount energy. To produce this energy, the neuron should first generate new mitochondria because pre-existing are likely unable provide sufficient acceleration in ATP production. Here, we demonstrate that mitochondrial biogenesis production...
Abstract Wolfram syndrome is a rare genetic disease caused by mutations in the WFS1 or CISD2 gene. A primary defect involves poor ER Ca 2+ handling, but how this disturbance leads to not known. The current study, performed neurons, most affected and disease-relevant cells, involving both genes, explains disturbed handling compromises mitochondrial function affects neuronal health. Loss of content impaired ER-mitochondrial contact sites WFS1- CISD2-deficient neurons associated with lower IP 3...
Variants in the SPATA5 gene were recently described a cohort of patients with global developmental delay, sensorineural hearing loss, seizures, cortical visual impairment and microcephaly. protein localizes predominantly mitochondria is proposed to be involved mitochondrial function brain processes. However no functional studies have been performed. This study describes five psychomotor microcephaly, epilepsy impairment, who thought clinically disease subsequent whole-exome sequencing...
During early development, neurons undergo complex morphological rearrangements to assemble into neuronal circuits and propagate signals. Rapid growth requires a large quantity of building materials, efficient intracellular transport also considerable amount energy. To produce this energy, the neuron should first generate new mitochondria because pre-existing are unlikely provide sufficient acceleration in ATP production. Here, we demonstrate that mitochondrial biogenesis production required...
SpringerPlus 2015, 4(Suppl 1):L1 MicroRNAs (miRNAs) are short, 22-25 nucleotide long transcripts that may suppress entire signaling pathways by interacting with the 3'-untranslated region (3'-UTR) of coding mRNA targets, interrupting translation and inducing degradation these targets.The 3'-UTRs brain compared to other tissues predict important roles for miRNAs.Supporting this notion, we found miRNAs co-evolved their target transcripts, non-coding pseudogenes miRNA recognition elements...
Wolfram syndrome (WS) 1 is a rare monogenic neurodegenerative disorder caused by mutations in the gene encoding WFS1. Knowledge of pathophysiology WS incomplete and to date, there no treatment available. Here, we describe early deviations renin-angiotensin-aldosterone system (RAAS) bradykinin pathway (kallikrein kinin system, KKS) observed rat model (Wfs1 KO) modulative effect glucagon-like peptide-1 receptor agonist liraglutide (LIR) anti-epileptic drug valproate (VPA), which have been...
<title>Abstract</title> Wolfram syndrome (WS) is a rare genetic disease caused by mutations in the WFS1 or CISD2 gene. A primary defect WS involves poor ER Ca<sup>2+</sup> handling, but how this disturbance leads to not known. The current study, performed isolated neurons, most affected and disease-relevant cells, involving both genes, explains disturbed handling compromises mitochondrial function affects neuronal health. Loss of content axons WFS1- CISD2-deficient neurons associated with...
SpringerPlus 2015, 4(Suppl 1):L1 MicroRNAs (miRNAs) are short, 22-25 nucleotide long transcripts that may suppress entire signaling pathways by interacting with the 3'-untranslated region (3'-UTR) of coding mRNA targets, interrupting translation and inducing degradation these targets.The 3'-UTRs brain compared to other tissues predict important roles for miRNAs.Supporting this notion, we found miRNAs co-evolved their target transcripts, non-coding pseudogenes miRNA recognition elements...