A5004570899- Cellular transport and secretion
- Trace Elements in Health
- Autophagy in Disease and Therapy
- Extracellular vesicles in disease
- Hereditary Neurological Disorders
- Endoplasmic Reticulum Stress and Disease
- Lysosomal Storage Disorders Research
- Mitochondrial Function and Pathology
- Polyamine Metabolism and Applications
- ATP Synthase and ATPases Research
- Heavy Metal Exposure and Toxicity
- Muscle Physiology and Disorders
- Advanced biosensing and bioanalysis techniques
- MicroRNA in disease regulation
- RNA Research and Splicing
- Alzheimer's disease research and treatments
- Neurogenetic and Muscular Disorders Research
- Dermatology and Skin Diseases
- Iron Metabolism and Disorders
- RNA modifications and cancer
- Asthma and respiratory diseases
- Photosynthetic Processes and Mechanisms
- Neurological diseases and metabolism
- Amyotrophic Lateral Sclerosis Research
- Glycosylation and Glycoproteins Research
Institute for Experimental Endocrinology and Oncology
2021-2025
National Research Council
2016-2025
National Academies of Sciences, Engineering, and Medicine
2018-2024
National Research Council
2023
Institute of Cell Biology and Neurobiology
2021
Institute of Protein Biochemistry
2016-2020
University of Naples Federico II
1976-2018
Istituto Neurologico Mediterraneo
2018
University Hospital of Bern
2018
University of Bern
2018
In several neurodegenerative diseases, axonal degeneration occurs before neuronal death and contributes significantly to patients’ disability. Hereditary spastic paraplegia (HSP) is a genetically heterogeneous condition characterized by selective of axons the corticospinal tracts fasciculus gracilis. HSP may therefore be considered an exemplary disease study local programs mediating degeneration. We have developed mouse model for autosomal recessive due mutations in SPG7 gene encoding...
In several neurodegenerative diseases, axonal degeneration occurs before neuronal death and contributes significantly to patients’ disability. Hereditary spastic paraplegia (HSP) is a genetically heterogeneous condition characterized by selective of axons the corticospinal tracts fasciculus gracilis. HSP may therefore be considered an exemplary disease study local programs mediating degeneration. We have developed mouse model for autosomal recessive due mutations in SPG7 gene encoding...
Lysosomal storage diseases are characterized by intracellular accumulation of metabolites within lysosomes. Recent evidence suggests that lysosomal impairs autophagy resulting in polyubiquitinated proteins and dysfunctional mitochondria, ultimately leading to apoptosis. We studied the relationship between lysosome impairment different pathways organelle function mucopolysaccharidosis VI, which is dermatan sulfate signs visceral skeletal but not cerebral involvement.We show storage, impaired...
TANGO1 (transport and Golgi organization 1) interacts with CTAGE5 COPII components Sec23/Sec24 recruits ERGIC-53 (endoplasmic reticulum [ER]–Golgi intermediate compartment 53)–containing membranes to generate a mega-transport carrier for export of collagens apolipoproteins from the ER. We now show that TANGO1, at ER, assembles in ring encircles components. The C-terminal, proline-rich domains molecules are initially tilted onto coats but appear be pushed apart as grows. These findings lend...
Autophagy is a self-degradative process responsible for the clearance of damaged or unnecessary cellular components. We have previously found that persistence dysfunctional organelles due to autophagy failure key event in pathogenesis COL6/collagen VI-related myopathies, and demonstrated reactivation proper autophagic flux rescues muscle defects Col6a1-null (col6a1(-/-)) mice. Here we show treatment with spermidine, naturally occurring nontoxic inducer, beneficial col6a1(-/-) Systemic...
During corticogenesis, late-born callosal projection neurons (CPNs) acquire their laminar position through glia-guided radial migration and then undergo final differentiation. However, the mechanisms controlling morphology of CPNs are poorly defined. Here, we show that in COUP-TFI mutant mice correctly specified, but delayed reaching cortical plate have morphological defects during migration. Interestingly, observed rate neuronal to normally follows a low-rostral high-caudal gradient,...
A-kinase anchoring proteins (AKAPs) transmit signals cues from seven-transmembrane receptors to specific sub-cellular locations. Mitochondrial AKAPs encoded by the Akap1 gene have been shown modulate mitochondrial function and reactive oxygen species (ROS) production in heart. Under conditions of hypoxia, AKAP121 undergoes proteolytic degradation mediated, at least part, E3 ubiquitin ligase Seven In-Absentia Homolog 2 (Siah2). In present study we hypothesized that might be crucial preserve...
Article13 September 2021Open Access Source DataTransparent process GRASP55 regulates intra-Golgi localization of glycosylation enzymes to control glycosphingolipid biosynthesis Prathyush Pothukuchi orcid.org/0000-0002-6242-2319 Institute Biochemistry and Cell Biology, National Research Council Italy, Rome, Italy Search for more papers by this author Ilenia Agliarulo These authors contributed equally work Marinella Pirozzi Riccardo Rizzo Domenico Russo Gabriele Turacchio Julian Nüchel...
Annexin A1 (ANXA1) is a calcium- and phospholipid-binding protein involved in broad range of cellular events. This study used molecular microscopy approaches to explore the role ANXA1 mouse myoblast C2C12 cell differentiation. We report that expression increases during differentiation down-regulation significantly inhibits process. expressed vivo both quiescent activated satellite cells highly localized migrate lumen regenerating fibers after an acute injury. Endogenous co-localizes with...
Degeneration of peripheral motor axons is a common feature several debilitating diseases including complicated forms hereditary spastic paraplegia. One such form caused by loss the mitochondrial energy-dependent protease paraplegin. Paraplegin-deficient mice display progressive degeneration in axonal tracts, characterized accumulation morphological abnormal mitochondria. We show that adenoassociated virus-mediated (AAV-mediated) intramuscular delivery paraplegin halted progression...
MitoAKAPs (mitochondrial A kinase anchoring proteins), encoded by the Akap1 gene, regulate multiple cellular processes governing mitochondrial homeostasis and cell viability. Although alterations have been associated to endothelial dysfunction, role of mitoAKAPs in vasculature is currently unknown. To test this, postischemic neovascularization, vascular function, arterial blood pressure were analyzed knockout mice (Akap1-/- ) their wild-type (wt) littermates. Primary cultures aortic cells...
Innate immune memory is characterized by a modulation in the magnitude with which innate cells such as monocytes and macrophages respond to potential dangers, subsequent previous exposure same or unrelated agents. In this study, we have examined capacity of gold nanoparticles (AuNP), are already use for therapeutic diagnostic purposes, modulate induced bacterial The induction was achieved vitro exposing human primary agents (lipopolysaccharide -LPS-, live Bacille Calmette-Guérin -BCG)...
Abstract Despite progress in the prevention and diagnosis of cancer, current technologies for tumor detection present several limitations including invasiveness, toxicity, inaccuracy, lengthy testing duration high cost. Therefore, innovative diagnostic techniques that integrate knowledge from biology, oncology, medicinal analytical chemistry are now quickly emerging attempt to address these issues. Following this approach, here we developed a paper-based electrochemical device detecting...
Thymic stromal lymphopoietin (TSLP), mainly expressed by epithelial cells, plays a central role in asthma. In humans, TSLP exists two variants: the long form (lfTSLP) and shorter isoform (sfTSLP). Macrophages (HLMs) mast cells (HLMCs) are close proximity human lung play key roles We evaluated early proteolytic effects of tryptase chymase released HLMCs on mass spectrometry. also investigated whether its fragments generated these enzymes induce angiogenic factor release from HLMs. Mass...
Current knowledge on the role of exosomal microRNA (miRNA) in canine leishmaniasis (CL), with particular regards to interaction between miR-122 and lipid alterations, is limited. The aim this study was isolate/characterize exosomes serum evaluate expression ten healthy leishmaniotic dogs. Serum were isolated using a polymer-based kit, ExoQuick® characterized by flow cytometry transmission electron microscopy, whereas miR-122-5p evaluated quantitative reverse-transcriptase polymerase chain...
Abstract The tumor microenvironment profoundly influences complexity, particularly in breast cancer, where cancer-associated fibroblasts play pivotal roles progression and therapy resistance. Extracellular vesicles are involved mediating communication within the TME, specifically highlighting their role promoting transformation of normal into fibroblasts. Recently, we identified an RNA aptamer, namely ex.50.T, that binds with remarkable affinity to extracellular shed from triple-negative...
Abstract Background Leukemia-secreted extracellular vesicles (EVs) carry biologically active cargo that promotes cancer-supportive mechanisms, including aberrant proliferative signaling, immune escape, and drug resistance. However, how antineoplastic drugs affect EV secretion sorting remains underexplored. Methods were isolated by Differential UltraCentrifugation, their miRNome proteomic profiling analyzed following treatment with SAHA (Vorinostat) in Acute Myeloid Leukemia (AML) Chronic...
Degeneration of peripheral motor axons is a common feature several debilitating diseases including complicated forms hereditary spastic paraplegia. One such form caused by loss the mitochondrial energy-dependent protease paraplegin. Paraplegin-deficient mice display progressive degeneration in axonal tracts, characterized accumulation morphological abnormal mitochondria. We show that adenoassociated virus–mediated (AAV-mediated) intramuscular delivery paraplegin halted progression...
Loss of cell adhesion and enhancement motility contribute to epithelial-to-mesenchymal transition during development. These processes are related a) rearrangement cell-cell cell-substrate molecules; b) cross talk between extra-cellular matrix internal cytoskeleton through focal molecules. Focal adhesions stringently regulated transient structures implicated in adhesion, spreading tissue Importantly, despite the extensive elucidation molecular composition adhesions, complex regulation their...
Inorganic diatomite nanoparticles (DNPs) have gained increasing interest as drug delivery systems due to their porous structure, long half-life, thermal and chemical stability. Gold (AuNPs) provide DNPs with intriguing optical features that can be engineered optimized for sensing applications. In this work, we combine gelatin stabilized AuNPs the development of an platform Galunisertib delivery. To improve DNP loading capacity, hybrid is capped shells thicknesses. Here, first time, full...
Significance Proteins are modified by many posttranslational modifications (PTMs) with crucial regulatory functions. A PTM attracting increasing interest is ADP-ribosylation, capable of altering cellular targets. We show that mono-ADP-ribosylation PARP12 the protein Golgin-97 regulates transport to plasma membrane a specific group functionally cargo proteins. shown be part cascade initiated PKD and involving direct phosphorylation activation PARP12. These events define, through...