Maria Lina Massimino

Impaired Neurosphere Formation as a Functional Readout of Aberrant EGFR Signaling Kinetics in Olfactory Neural Progenitors From Patients With Schizophrenia

Tommaso Toffanin, Mario A. Pagano, Carlo Idotta, Mauro Salvi et autres

The Epidermal Growth Factor Receptor (EGFR) is a key regulator of neurodevelopment, controlling the proliferation, differentiation, and self-renewal of neural stem and progenitor cells. Dysregulated EGFR signaling has been implicated in schizophrenia, a neurodevelopmental disorder associated with abnormal cortical maturation. We investigated …

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Extracellular vesicles as novel mediators of pathology in Anderson–Fabry disease

Anna Olena Kalmykova, Maria Lina Massimino, Monica Vettore, Giorgia Gugelmo et autres

Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder caused by GLA mutations that reduce α-galactosidase A activity, leading to systemic accumulation of globotriaosylceramide (Gb3). Increasing evidence indicates that extracellular vesicles (EVs) contribute to pathogenic signal dissemination in lysosomal storage disorders. We …

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Extracellular vesicles as new mediators of pathology in Anderson Fabry disease

Livia Lenzini, Maria Lina Massimino, Anna Olena Kalmykova, Monica Vettore et autres

#2556 Exploring exosome-mediated effects on renal pathology in Anderson Fabry disease

Livia Lenzini, Maria Lina Massimino, Monica Vettore, Elisabetta Iori et autres

Abstract Background and Aims Despite substantial progress in understanding the molecular basis of genetic lysosomal storage diseases, the systemic mechanisms linking lysosomal defects in specific cells to widespread organ damage remain unclear, particularly in Anderson-Fabry disease (AFD). This project introduces a novel …

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Targeting Neuromuscular Junction Regeneration is a Therapeutic Strategy in ALS

Samuele Negro, Chiara Baggio, Giorgia D’Este, F Fabris et autres

Abstract Instability and denervation of the neuromuscular junction (NMJ) are early events in Amyotrophic Lateral Sclerosis (ALS), likely reflecting a progressive decline in the regenerative capacity of motor neurons (MNs) and their environment. To investigate this, we evaluated NMJ regeneration throughout disease …

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Restoration of myogenesis in ALS-myocytes through miR-26a-5p-mediated Smad4 inhibition and its impact on motor neuron development

Caterina Peggion, Raphael Severino Bonadio, Roberto Stella, Silvia Scalabrin et autres

Amyotrophic lateral sclerosis (ALS) is the most common adult-onset paralytic disorder, characterized primarily by a progressive loss of motor neurons (MNs) in which degeneration skeletal muscle involvement has been demonstrated. Skeletal muscle is a plastic tissue that responds to insults through proliferation …

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Structural Integrity of Nucleolin Is Required to Suppress TDP-43-Mediated Cytotoxicity in Yeast and Human Cell Models

Caterina Peggion, Maria Lina Massimino, Daniel Pereira, Sara Granuzzo et autres

The Transactivating response (TAR) element DNA-binding of 43 kDa (TDP-43) is mainly implicated in the regulation of gene expression, playing multiple roles in RNA metabolism. Pathologically, it is implicated in amyotrophic lateral sclerosis and in a class of neurodegenerative diseases broadly going …

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Essential Oil Molecules Can Break the Loop of Oxidative Stress in Neurodegenerative Diseases

Enzo Spisni, Maria Chiara Valerii, Maria Lina Massimino

Essential oils (EOs) are mixtures of volatile compounds, extracted from aromatic plants, with multiple activities including antioxidant and anti-inflammatory ones. EOs are complex mixtures easy to find on the market and with low costs. In this mini narrative review, we have collected …

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Secreted Metabolome of ALS-Related hSOD1(G93A) Primary Cultures of Myocytes and Implications for Myogenesis

Roberto Stella, Raphael Severino Bonadio, Stefano Cagnin, Roberta Andreotti et autres

Amyotrophic lateral sclerosis (ALS) is a motor neuron (MN) disease associated with progressive muscle atrophy, paralysis, and eventually death. Growing evidence demonstrates that the pathological process leading to ALS is the result of multiple altered mechanisms occurring not only in MNs but …

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Pllans−II: Unveiling the Action Mechanism of a Promising Chemotherapeutic Agent Targeting Cervical Cancer Cell Adhesion and Survival Pathways

Alejandro Montoya‐Gómez, Fiorella Tonello, Barbara Spolaore, Maria Lina Massimino et autres

Despite advances in chemotherapeutic drugs used against cervical cancer, available chemotherapy treatments adversely affect the patient’s quality of life. For this reason, new molecules from natural sources with antitumor potential and few side effects are required. In previous research, Pllans−II, a phospholipase …

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Nucleolin: a cell portal for viruses, bacteria, and toxins

Fiorella Tonello, Maria Lina Massimino, Caterina Peggion

The main localization of nucleolin is the nucleolus, but this protein is present in multiple subcellular sites, and it is unconventionally secreted. On the cell surface, nucleolin acts as a receptor for various viruses, some bacteria, and some toxins. Aim of this …

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SOD1 in ALS: Taking Stock in Pathogenic Mechanisms and the Role of Glial and Muscle Cells

Caterina Peggion, Valeria Scalcon, Maria Lina Massimino, Kelly Nies et autres

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by the loss of motor neurons in the brain and spinal cord. While the exact causes of ALS are still unclear, the discovery that familial cases of ALS are related to mutations …

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