A5029951427- Mitochondrial Function and Pathology
- Autophagy in Disease and Therapy
- Metabolism and Genetic Disorders
- Alzheimer's disease research and treatments
- Neuroscience and Neuropharmacology Research
- Genetic Neurodegenerative Diseases
- Metalloenzymes and iron-sulfur proteins
- ATP Synthase and ATPases Research
- RNA modifications and cancer
- Epigenetics and DNA Methylation
- Amyotrophic Lateral Sclerosis Research
- Tryptophan and brain disorders
- Congenital heart defects research
- Adipose Tissue and Metabolism
- Endoplasmic Reticulum Stress and Disease
- Cholinesterase and Neurodegenerative Diseases
- Retinal Development and Disorders
- Metal-Organic Frameworks: Synthesis and Applications
- Parkinson's Disease Mechanisms and Treatments
- Genetics and Neurodevelopmental Disorders
- Medical Imaging and Pathology Studies
- Genomics and Rare Diseases
- Cancer-related gene regulation
Johannes Gutenberg University Mainz
2020-2024
University Medical Center of the Johannes Gutenberg University Mainz
2020-2024
Institute for Molecular Medicine
2021-2024
University Medical Center Hamburg-Eppendorf
2020
Universität Hamburg
2020
Universidad Nacional de Colombia
2014
Abstract One mechanism of particular interest to regulate mRNA fate post-transcriptionally is modification. Especially the extent m 1 A methylation highly discussed due methodological differences. However, one single site in mitochondrial ND5 was unanimously reported by different groups. a subunit complex I respiratory chain. It considered essential for coupling oxidation and proton transport. Here we demonstrate that this might be involved pathophysiology Alzheimer’s disease (AD)....
Ion fluxes across the inner mitochondrial membrane control volume, energy production, and apoptosis. TMBIM5, a highly conserved protein with homology to putative pH-dependent ion channels, is involved in maintenance of cristae architecture, ATP Here, we demonstrate that overexpressed TMBIM5 can mediate calcium uptake. Under steady-state conditions, loss results increased potassium reduced proton levels matrix caused by attenuated exchange these ions. To identify vivo consequences...
Ion transport within mitochondria influences their structure, energy production, and cell death regulation. TMBIM5, a conserved calcium/proton exchanger in the inner mitochondrial membrane, contributes to ATP synthesis, apoptosis The relationship of TMBIM5 with calcium uniporter complex formed by MCU, MICU1-3, EMRE remains undefined. We generated Tmbim5-deficient Drosophila that exhibit disrupted cristae architecture, premature permeability transition pore opening, reduced uptake, swelling -...
Charcot-Marie-Tooth (CMT) disease 4A is an autosomal-recessive polyneuropathy caused by mutations of ganglioside-induced differentiation-associated protein 1 (GDAP1), a putative glutathione transferase, which affects mitochondrial shape and alters cellular Ca2+ homeostasis. Here, we identify the underlying mechanism. We found that patient-derived motoneurons GDAP1 knockdown SH-SY5Y cells display two phenotypes: more tubular mitochondria metabolism characterized glutamine dependence fewer...
Chronic stress has the potential to impair health and may increase vulnerability for psychiatric disorders. Emerging evidence suggests that specific neurometabolic dysfunctions play a role herein. In mice, chronic social defeat (CSD) reduces cerebral glucose uptake despite hyperglycemia. We hypothesized this metabolic decoupling would be reflected by changes in contact sites between mitochondria endoplasmic reticulum, important intracellular nutrient sensors, signaling hubs. thus analyzed...
Parkinson’s disease (PD) is characterized by the progressive loss of dopaminergic neurons in substantia nigra midbrain. Familial cases PD are often caused mutations PTEN-induced kinase 1 (PINK1) and ubiquitin ligase Parkin, both pivotal maintaining mitochondrial quality control. CISD1, a homodimeric iron-sulfur-binding protein, major target Parkin-mediated ubiquitination. We here discovered heightened propensity CISD1 to form dimers Pink1 mutant flies from PINK1 mutation patients. The dimer...
Abstract Presenilin 1 (PS1) mutations are the most common cause of familial Alzheimer’s disease (FAD). PS1 also plays a role in cellular processes such as calcium homeostasis and autophagy. We hypothesized that mutant presenilins increase vulnerability to stress. stably expressed human PS1, PS1E280A PS1Δ9 mouse neuroblastoma N2a cells. examined early signs stress different conditions: endoplasmic reticulum (ER) stress, overload, oxidative Aβ 1–42 oligomers toxicity. Additionally, we induced...
Synaptic signaling depends on ATP generated by mitochondria. Dysfunctional mitochondria shift the redox balance towards a more oxidative environment. Due to extensive connectivity, striatum is especially vulnerable mitochondrial dysfunction. We found that neuronal calcium-binding protein 2 (NECAB2) plays role in striatal function and homeostasis. NECAB2 predominantly endosomal which partially colocalizes with This colocalization enhanced mild stress. Global knockout of Necab2 mouse results...
Abstract One mechanism of particular interest to regulate mRNA fate post-transcriptionally is modification. Especially the extent m 1 A methylation highly discussed due methodological differences. However, one single site in mitochondrial ND5 was unanimously reported by different groups. a subunit complex I respiratory chain. It considered essential for coupling oxidation and proton transport. Here we demonstrate that this might be involved pathophysiology Alzheimer’s disease (AD)....
Abstract Parkinson’s disease (PD) is characterized by the progressive loss of dopaminergic neurons in substantia nigra midbrain. Familial cases PD are often caused mutations PTEN-induced kinase 1 (PINK1) and ubiquitin ligase Parkin, both pivotal maintaining mitochondrial quality control. CISD1, a homodimeric iron-sulfur-binding protein, major target Parkin-mediated ubiquitination. We here discovered heightened propensity CISD1 to form dimers Pink1 mutant flies from PINK1 mutation patients....
Abstract Synaptic signaling depends on ATP generated by mitochondria. Due to extensive connectivity, the striatum is especially vulnerable mitochondrial dysfunction and thus requires efficient quality control repair. We found that global knockout of neuronal calcium-binding protein 2 (NECAB2) in mouse causes loss striatal synapses behavioral phenotypes related such as reduced motivation altered sensory gating. Striatal mitochondria from Necab2 mice are more abundant smaller. They...