A5077672709- Neurogenetic and Muscular Disorders Research
- Amyotrophic Lateral Sclerosis Research
- RNA Research and Splicing
- MXene and MAX Phase Materials
- Muscle Physiology and Disorders
- 3D Printing in Biomedical Research
- Ferroelectric and Negative Capacitance Devices
- Medical Research and Treatments
- Extracellular vesicles in disease
- Additive Manufacturing and 3D Printing Technologies
- RNA modifications and cancer
- Bone Tissue Engineering Materials
- Microfluidic and Bio-sensing Technologies
- Nanofabrication and Lithography Techniques
- Electrospun Nanofibers in Biomedical Applications
- Silk-based biomaterials and applications
- Congenital Anomalies and Fetal Surgery
- biodegradable polymer synthesis and properties
University College London
2022-2024
Fondazione IRCCS Istituto Neurologico Carlo Besta
2020-2024
The Francis Crick Institute
2022-2024
University of Milano-Bicocca
2020-2021
Spinal muscular atrophy (SMA) is an autosomal recessive disorder caused by mutations in survival motor neuron (SMN) 1 gene, resulting a truncated SMN protein responsible for degeneration of brain stem and spinal neurons. The paralogous SMN2 gene partially compensates full-length production, mitigating the phenotype. Antisense oligonucleotide nusinersen (Spinraza®) enhances expression. involved RNA metabolism biogenesis microRNA (miRNA), key expression modulators, whose dysregulation...
Background and objectives Multisystem involvement in spinal muscular atrophy (SMA) is gaining prominence since different therapeutic options are emerging, making the way for new SMA phenotypes consequent challenges clinical care. Defective immune organs have been found preclinical models of SMA, suggesting an system disease. However, state patients has not investigated so far. Here, we aimed to evaluate innate adaptive immunity pattern type 1 3 patients, before after nusinersen treatment....
Motor neuron diseases (MNDs) are neurodegenerative disorders characterized by upper and/or lower MN loss. MNDs include amyotrophic lateral sclerosis (ALS), spinal muscular atrophy (SMA), and bulbar (SBMA). Despite variability in onset, progression, genetics, they share a common skeletal muscle involvement, suggesting that it could be primary site for MND pathogenesis. Due to the key role of muscle-specific microRNAs (myomiRs) development, real-time PCR we investigated expression miR-206,...
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease affecting motor neurons. Considerable evidence indicates that early skeletal muscle atrophy plays crucial role in the pathogenesis, leading to an altered muscle-motor neuron crosstalk that, turn, may contribute degeneration. Currently, there no effective treatment for ALS, highlighting need dig deeper into pathological mechanisms developing innovative therapeutic strategies. FM19G11 novel drug able modulate global...
Cell culture devices, such as microwells and microfluidic chips, are designed to increase the complexity of cell-based models while retaining control over conditions have become indispensable platforms for biological systems modelling. From microtopography, microwells, plating larger constructs live imaging chamber slides, a wide variety devices with different geometries in biology laboratories. However, their application projects is increasing exponentially, due combination techniques,...
Cell microencapsulation has been utilized for years as a means of cell shielding from the external environment while facilitating transport gases, general metabolites, and secretory bioactive molecules at once. In this light, hydrogels may support structural integrity functionality encapsulated biologics whereas ensuring viability function releasing potential therapeutic factors once in situ. work, we describe straightforward strategy to fabricate silk fibroin (SF) microgels (µgels)...
Abstract Cell culture devices, such as microwells and microfluidic chips, are designed to increase the complexity of cell-based models whilst retaining control over conditions have become indispensable platforms for biological systems modelling. From microtopography, microwells, plating devices larger constructs specific applications live imaging chamber slides, a wide variety with different geometries in biology laboratories. However, while their application projects is increasing...
Amyotrophic lateral sclerosis (ALS) is characterized by the progressive, irreversible loss of upper and lower motor neurons (UMNs, LMNs). MN axonal dysfunctions are emerging as relevant pathogenic events since early ALS stages. However, exact molecular mechanisms leading to axon degeneration in still need be clarified. MicroRNA (miRNA) dysregulation plays a critical role pathogenesis neuromuscular diseases. These molecules represent promising biomarkers for these conditions their expression...