A5044476488- Neurological diseases and metabolism
- RNA regulation and disease
- Neuroscience and Neuropharmacology Research
- Photoreceptor and optogenetics research
- Trace Elements in Health
- Porphyrin Metabolism and Disorders
- Iron Metabolism and Disorders
- Retinal Development and Disorders
- Genetics and Neurodevelopmental Disorders
- Mitochondrial Function and Pathology
- Hereditary Neurological Disorders
- Neurogenesis and neuroplasticity mechanisms
- Neuroscience and Neural Engineering
- Lipid Membrane Structure and Behavior
- Cellular transport and secretion
- Amyotrophic Lateral Sclerosis Research
- Glioma Diagnosis and Treatment
- Axon Guidance and Neuronal Signaling
- Multiple Sclerosis Research Studies
- Advanced Nanomaterials in Catalysis
- ATP Synthase and ATPases Research
- Supramolecular Self-Assembly in Materials
- Image Enhancement Techniques
- Protein Kinase Regulation and GTPase Signaling
- Cell Image Analysis Techniques
Vita-Salute San Raffaele University
2010-2024
Istituti di Ricovero e Cura a Carattere Scientifico
2020-2022
Istituto di Ricovero e Cura a Carattere Scientifico San Raffaele
2020-2022
Neuroscience Institute
2020-2022
San Raffaele University of Rome
2014-2021
Politecnico di Milano
2002
Istituto Nazionale di Fisica Nucleare, Sezione di Milano
1995
The endoplasmic reticulum (ER) is involved in many functions, including protein folding, redox homeostasis, and Ca(2+) storage signaling. To perform these multiple tasks, the ER composed of distinct, specialized subregions, amongst which mitochondrial-associated membranes (MAM) emerge as key signaling hubs. How functions are integrated with one another living cells remains unclear.Here we show that Ero1α, a controller oxidative folding enriched MAM regulates fluxes. Downregulation Ero1α by...
Neuroferritinopathy (NF) is a movement disorder caused by alterations in the L-ferritin gene that generate cytosolic free iron. NF unique pathophysiological model for determining direct consequences of cell iron dysregulation. We established lines induced pluripotent stem cells from fibroblasts two patients and one isogenic control obtained CRISPR/Cas9 technology. fibroblasts, neural progenitors, neurons exhibited presence increased iron, which was also detectable as: ferritin aggregates,...
Abstract Neurodegeneration associated with defective pantothenate kinase-2 (PKAN) is an early-onset monogenic autosomal-recessive disorder. The hallmark of the disease massive accumulation iron in globus pallidus brain region patients. PKAN caused by mutations PANK2 gene encoding mitochondrial enzyme kinase-2, whose function to catalyze first reaction CoA biosynthetic pathway. To date, way which this alteration leads has not been elucidated. Starting from previously obtained hiPS clones, we...
The excitation functions for production of many residues in the fusion $^{12}\mathrm{C}$ with $^{181}\mathrm{Ta}$ and $^{197}\mathrm{Au}$ $^{16}\mathrm{O}$ $^{165}\mathrm{Ho}$ have been measured at incident energies varying from Coulomb barrier up to about 10 MeV/nucleon. cross sections these ions without fission deduced they show that such a process may only occur projectile angular momenta smaller than critical value. individual reactions provide evidence emission preequilibrium nucleons...
Although patients with chronic renal failure are increasing worldwide, many aspects of kidney biology remain to be elucidated. Recent research has uncovered several molecular properties the glomerular filtration barrier, in which podocytes, highly differentiated, ramified cells that enwrap basement membrane, have been reported mainly responsible for filter's selectivity. We previously described podocytes express Rab3A, a GTPase restricted cell types capable regulated exocytosis, such as...
The identification of biomaterials which promote neuronal maturation up to the generation integrated neural circuits is fundamental for modern neuroscience. development arises from complex maturative processes regulated by poorly understood signalling events, often guided extracellular matrix (ECM). Here we report that nanostructured zirconia surfaces, produced supersonic cluster beam deposition nanoparticles and characterised ECM-like nanotopographical features, can direct networks....
Abstract Disease models of neurodegeneration with brain iron accumulation (NBIA) offer the possibility to explore relationship between dyshomeostasis and neurodegeneration. We analyzed hiPS-derived astrocytes from PANK2-associated (PKAN), an NBIA disease characterized by progressive high in globus pallidus. Previous data indicated that PKAN exhibit alterations metabolism, general impairment constitutive endosomal trafficking, mitochondrial dysfunction acquired neurotoxic features. Here, we...
Ideally, elucidating the role of specific brain circuits in animal behavior would require ability to measure activity at all involved synapses, possibly with unrestricted field view, thus even those boutons deeply located into brain. Here, we introduce and validate an efficient scheme reporting synaptic vesicle cycling vivo. This is based on SynaptoZip, a genetically encoded molecule deploying vesicular lumen bait moiety designed capture upon exocytosis labeled alien peptide, Synbond. The...
Pantothenate Kinase-associated Neurodegeneration (PKAN) belongs to a wide spectrum of diseases characterized by brain iron accumulation and extrapyramidal motor signs. PKAN is caused mutations in PANK2, encoding the mitochondrial pantothenate kinase 2, which first enzyme biosynthesis Coenzyme A. We established glutamatergic neurons starting from previously developed Induced Pluripotent Stem Cells (iPSCs). Results obtained inductively coupled plasma mass spectrometry indicated higher amount...
Key points The mechanisms of action anaesthetics on the living brain are still poorly understood. In this respect, analysis differential effects spontaneous and sensory‐evoked cortical activity might provide important novel cues. Here we show that anaesthetic sevoflurane strongly silences but potentiates in a dose‐ frequency‐dependent manner visual response. Such enhancement arises from linear scaling by power‐law relation between light intensity fingerprint suggests circuit silencing can...
GDI1 gene encodes for αGDI, a protein controlling the cycling of small GTPases, reputed to orchestrate vesicle trafficking. Mutations in human are responsible intellectual disability (ID). In mice with ablated Gdi1, model ID, impaired working and associative short-term memory was recorded. This cognitive phenotype worsens if deletion αGDI expression is restricted neurons. However, whether astrocytes, key homeostasis providing neuroglial cells, supporting neurons via aerobic glycolysis,...
COASY protein-associated neurodegeneration (CoPAN) is a rare but devastating genetic autosomal recessive disorder of inborn error CoA metabolism, which shares with pantothenate kinase-associated (PKAN) similar features, such as dystonia, parkinsonian traits, cognitive impairment, axonal neuropathy, and brain iron accumulation. These two disorders are part the big group neurodegenerations accumulation (NBIA) for no effective treatment available at moment. To date, lack mammalian model, fully...
Abstract The adult brain retains over life endogenous neural stem/precursor cells (eNPCs) within the subventricular zone (SVZ). Whether or not these exert physiological functions is still unclear. In present work, we provide evidence that SVZ-eNPCs tune structural, electrophysiological, and behavioural aspects of striatal function via secretion insulin-like growth factor binding protein-like 1 (IGFBPL1). mice, selective ablation abrogation IGFBPL1 determined an impairment medium spiny neuron...
A fundamental question in vision neuroscience is how parallel processing of Retinal Ganglion Cell (RGC) signals integrated at the level visual thalamus. It well-known that ON-OFF pathways generate output from retina are conveyed to dorsal lateral geniculate nucleus (dLGN). However, it unclear these distribute onto thalamic cells and two interact. Here, by electrophysiological recordings c-Fos expression analysis, we characterized effects pharmacological manipulations retinal circuit aimed...
In many neurodegenerative disorders, including amyotrophic lateral sclerosis (ALS), synaptic alterations precede the demise of neuronal cell, making synapses a useful vantage point from which to monitor onset and progression clinical signs pathological changes. While murine models ALS display features in common with picture observed patients, corticospinal tract (CST) involvement is usually less severe mice than humans. this paper we describe characterization new conditional transgenic line...
PKAN disease is caused by mutations in the PANK2 gene, encoding mitochondrial enzyme pantothenate kinase 2, catalyzing first and key reaction Coenzyme A (CoA) biosynthetic process. This disorder characterized progressive neurodegeneration excessive iron deposition brain. The pathogenic mechanisms of are still unclear, available therapies only symptomatic. Although accumulation a hallmark PKAN, its relationship with CoA dysfunction not clear. We have previously developed hiPS-derived...
Calcium (Ca(2+)) waves and Ca(2+) oscillations within cells initiate a wide range of physiological processes including control cell signaling, gene expression, secretion, migration. A thorough analysis in glial provides information not only about the subcellular location signaling processing events but also nonneuronal or intercellular pathways, their timing, routes, spatial domains, coordination. In this study, three novel image methods have been applied to study dynamics cells. These bring...
One of the main problems that affect fluoroscopic imaging is difficulty in coupling recorded activity with morphological information. The comprehension fluorescence events relationship internal structure cell can be very difficult. At this purpose, we developed a new method able to maximize movie quality. (Maximum Intensity Enhancement, MIE) works as follow: considering all frames compose movie, algorithm extracts, for each pixel matrix, maximal brightness value assumed along frames. Such...
Abstract The adult brain retains endogenous neural stem/precursors cells (eNPCs) in two major neurogenic niches, the subgranular zone (SGZ) hippocampus and subventricular (SVZ). While humans rodents SGZ contributes to memory functions by generating new neurons that integrate into hippocampal circuitry, role of eNPCs SVZ is less clear. SVZ-eNPCs contribute generation fated olfactory bulb (OB) but not where they are thought striatal neurogenesis as a result tissue damage. Here, we show mice...
In the last decade new ideas were born about temporal and spatial dynamics of intercellular calcium waves in astrocytes. this paper we introduce a approach to analyze ways which astrocytes communicate cultures. We present method describe propagation Ca(2+) vitro technique compare activity different cells vivo under stimulation conditions. The proposed resulted be an interesting way distinguish astrocyte clusters, can related communication characteristics network.
An effective motion compensated image interpolation technique is presented. The proposed algorithm has been developed for the of missing frames in an sequence and it based on two principal elements. first a region-based estimation representation technique, which combines, each known image, approximate initial field estimate over-segmentation (obtained using both luminance chrominance information) to produce very accurate affine-model regularized field. uses robust identification parameters...