A5060274257- Mitochondrial Function and Pathology
- Metabolism and Genetic Disorders
- ATP Synthase and ATPases Research
- Amyotrophic Lateral Sclerosis Research
- Thermal and Kinetic Analysis
- RNA and protein synthesis mechanisms
- Inorganic and Organometallic Chemistry
- Metal complexes synthesis and properties
- Alzheimer's disease research and treatments
- Parkinson's Disease Mechanisms and Treatments
- Cholinesterase and Neurodegenerative Diseases
- Genetic Neurodegenerative Diseases
- Cancer, Hypoxia, and Metabolism
- Chemical Thermodynamics and Molecular Structure
- Ion channel regulation and function
- Neuroinflammation and Neurodegeneration Mechanisms
- Autophagy in Disease and Therapy
- Trace Elements in Health
- GABA and Rice Research
- Photosynthetic Processes and Mechanisms
- RNA modifications and cancer
- Extracellular vesicles in disease
- Neurogenetic and Muscular Disorders Research
- Synthesis and biological activity
- Molecular Sensors and Ion Detection
University of Catania
2016-2025
Technobiochip (Italy)
2021
Istituto Nazionale Biostrutture e Biosistemi
2016
Institute of Biostructure and Bioimaging
2014-2016
The University of Texas Health Science Center at San Antonio
2010-2013
Sapienza University of Rome
1983-2000
Accumulation of tau is a critical event in several neurodegenerative disorders, collectively known as tauopathies, which include Alzheimer's disease and frontotemporal dementia. Pathological hyperphosphorylated aggregates to form neurofibrillary tangles. The molecular mechanisms leading accumulation remain unclear more needs be done elucidate them. Age major risk factor for all suggesting that changes contributing the aging process may facilitate represent common across different...
Reducing the mammalian target of rapamycin (mTOR) activity increases lifespan and health span in a variety organisms. Alterations protein homeostasis mTOR signaling have been reported several neurodegenerative disorders, including Alzheimer disease (AD); however, causes such deregulations remain elusive. Here, we show that are increased cell lines stably transfected with mutant amyloid precursor (APP) brains 3xTg-AD mice, an animal model AD. In addition, can be reduced to wild type levels by...
Unraveling the role of VDAC3 within living cells is challenging and still requires a definitive answer. Unlike VDAC1 VDAC2, outer mitochondrial membrane porin 3 exhibits unique biophysical features that suggest unknown cellular functions. Electrophysiological studies on carrying selective cysteine mutations mass spectrometry data about redox state such sulfur containing amino acids are consistent with putative involvement isoform in ROS homeostasis. Here, we thoroughly examined this issue...
// Simona Reina 1,2* , Vanessa Checchetto 3,4,5,* Rosaria Saletti 6 Ankit Gupta 7,* Deepti Chaturvedi Carlo Guardiani 8 Francesca Guarino 1,2 Mariano Andrea Scorciapino 9 Magrì Salvatore Foti Matteo Ceccarelli 8,10,** Angela Anna Messina 11,12,** Radhakrishnan Mahalakshmi 7,** Ildiko Szabo 3,4,** and Vito De Pinto 1 Department of Biomedicine Biotechnology BIOMETEC, Section Biology Genetics, University Catania, Italy 2 National Institute for Biomembranes Biosystems, 3 Biology, Padova, 4 CNR...
MPP+ is the active metabolite of MPTP, a molecule structurally similar to herbicide Paraquat, known injure dopaminergic neurons nigrostriatal system in Parkinson’s disease models. Within cells, accumulates mitochondria where it inhibits complex I electron transport chain, resulting ATP depletion and neuronal impairment/death. So far, recognized as valuable tool mimic degeneration various cell lines. However, despite large number studies, detailed characterization mitochondrial respiration...
Extracellular vesicles (EVs) are emerging as powerful players in cell-to-cell communication both healthy and diseased brain. In Parkinson's disease (PD)-characterized by selective dopaminergic neuron death ventral midbrain (VMB) degeneration of their terminals striatum (STR)-astrocytes exert dual harmful/protective functions, with mechanisms not fully elucidated. Here, this study shows that astrocytes from the VMB-, STR-, VMB/STR-depleted brains release a population small EVs region-specific...
Abstract Mitochondrial dysfunction and the loss of mitophagy, aimed at recycling irreversibly damaged organelles, contribute to onset amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disease affecting spinal cord motor neurons. In this work, we showed that reduction mitochondrial respiration, exactly oxygen flows linked ATP production maximal capacity, correlates with appearance most common ALS symptoms in transgenic mouse model expressing SOD1 G93A mutant. This is result equal...
The voltage-dependent anion-selective channel isoform 1 (VDAC1) is a pivotal component in cellular metabolism and apoptosis with prominent role many cancer types, offering unique therapeutic intervention point. Through an
Background/Aims: Voltage-dependent anion channels (VDAC), also known as eukaryotic porins, are located in the outer mitochondrial membrane and allow flux of ions small metabolites. While pore-forming ability recombinant VDAC1 VDAC2 has been extensively studied during last decades, a clear-cut ion conducting channel activity not assigned to VDAC3 isoform. Methods : Electrophysiological characterization protein purified refolded was obtained after incorporation into planar lipid bilayers....
Abstract Superoxide Dismutase 1 mutants associate with 20–25% of familial Amyotrophic Lateral Sclerosis (ALS) cases, producing toxic aggregates on mitochondria, notably in spinal cord. The Voltage Dependent Anion Channel isoform (VDAC1) the outer mitochondrial membrane is a docking site for SOD1 G93A mutant ALS mice and physiological receptor Hexokinase I (HK1), which poorly expressed mouse Our results demonstrate that HK1 competes binding VDAC1, suggesting cord available HK1-binding sites...
Voltage-dependent anion channel (VDAC) is the major pathway for transport of ions and metabolites across mitochondrial outer membrane. Among three known mammalian VDAC isoforms, VDAC3 least characterized, but unique functional roles have been proposed in cellular animal models. Yet, a high-sequence similarity between VDAC1 indicative similar pore-forming structure. Here, we conclusively show that forms stable, highly conductive voltage-gated channels that, much like VDAC1, are weakly...
ABSTRACT Mitochondrial dysfunction has been implicated in a broad range of age‐related pathologies and proposed as causative factor Alzheimer's disease (AD). Analysis post‐mortem brains from AD patients showed increased levels Voltage‐dependent anion‐selective channel 1 (VDAC1) the dystrophic neurites surrounding amyloid‐β (Aβ) deposits, suggesting direct association between VDAC1 mitochondrial toxicity. is most abundant pore‐forming protein outer membrane and, channel, it plays pivotal role...
Mutations in superoxide dismutase (SOD1) are the second most common cause of familial amyotrophic lateral sclerosis (ALS), a progressive neurodegenerative disease characterized by loss motor neurons brain and spinal cord. SOD1 neurotoxicity has been attributed to aberrant accumulation misfolded SOD1, which its soluble form binds intracellular organelles, such as mitochondria ER, disrupting their functions. Here, we demonstrate that mutant specifically N-terminal domain voltage-dependent...
Voltage-Dependent Anion-selective Channel isoform 1 (VDAC1) is the most abundant of outer mitochondrial membrane (OMM) porins and principal gate for ions metabolites to from organelle. VDAC1 also involved in a number additional functions, such as regulation apoptosis. Although protein not directly respiration, its deletion yeast triggers complete rewiring whole cell metabolism, with inactivation main functions. In this work, we analyzed detail impact knockout on respiration near-haploid...
In higher eukaryotes three different VDAC genes encode homologous proteins which do not show the same activity.
Mutations in Cu/Zn Superoxide Dismutase (SOD1) gene represent one of the most common causes amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disorder that specifically affects motor neurons (MNs). The dismutase-active SOD1 G93A mutant is responsible for formation toxic aggregates onto mitochondrial surface, using Voltage-Dependent Anion Channel 1 (VDAC1) as an anchor point to organelle. VDAC1 master regulator cellular bioenergetics and by binding hexokinases (HKs) it controls...