A5043890990- Amyotrophic Lateral Sclerosis Research
- Neurogenetic and Muscular Disorders Research
- Neuroinflammation and Neurodegeneration Mechanisms
- Parkinson's Disease Mechanisms and Treatments
- Immune cells in cancer
- Immune Response and Inflammation
- Gut microbiota and health
- biodegradable polymer synthesis and properties
- Histone Deacetylase Inhibitors Research
- Muscle Physiology and Disorders
- Nerve injury and regeneration
- Neurological diseases and metabolism
- RNA and protein synthesis mechanisms
- RNA Research and Splicing
- Tryptophan and brain disorders
- Genetic Neurodegenerative Diseases
- Alzheimer's disease research and treatments
- Cholinesterase and Neurodegenerative Diseases
- Welding Techniques and Residual Stresses
- RNA modifications and cancer
- Spinal Cord Injury Research
Mario Negri Institute for Pharmacological Research
2014-2025
Istituti di Ricovero e Cura a Carattere Scientifico
2017-2023
Abstract Microglial activation plays central roles in neuroinflammatory and neurodegenerative diseases. Positron emission tomography (PET) targeting 18 kDa Translocator Protein (TSPO) is widely used for localising inflammation vivo, but its quantitative interpretation remains uncertain. We show that TSPO expression increases activated microglia mouse brain disease models does not change a non-human primate model or common human describe genetic divergence the gene promoter, consistent with...
Amyotrophic lateral sclerosis is heterogeneous with high variability in the speed of progression even cases a defined genetic cause such as superoxide dismutase 1 (SOD1) mutations. We reported that SOD1G93A mice on distinct backgrounds (C57 and 129Sv) show consistent phenotypic differences disease life-span are not explained by human SOD1 transgene copy number or burden mutant protein within nervous system. aimed to compare gene expression profiles motor neurons from these two mouse strains...
Increasing evidence suggests that the immune system has a beneficial role in progression of amyotrophic lateral sclerosis (ALS) although mechanism remains unclear. Recently, we demonstrated motor neurons (MNs) C57SOD1G93A mice with slow disease activate molecules classically involved cross-talk system. This happens lot less 129SvSOD1G93A which, while expressing same amount transgene, had faster and earlier axonal damage. The present study investigated whether how response is preservation...
The major histocompatibility complex I (MHCI) is a key molecule for the interaction of mononucleated cells with CD8+T lymphocytes. We previously showed that MHCI upregulated in spinal cord microglia and motor axons transgenic SOD1G93A mice.To assess role disease, we examined mice crossbred β2 microglobulin-deficient mice, which express little if any on cell surface are defective CD8+ T cells.The lack sciatic nerve affects axon stability, anticipating muscle atrophy disease onset. In...
Amyotrophic lateral sclerosis (ALS) is a heterogeneous disease in terms of onset and progression rate. This may account for therapeutic clinical trial failure. Transgenic SOD1G93A mice on C57 or 129Sv background have slow fast rate, mimicking the variability observed patients. Based evidence inferring active influence skeletal muscle ALS pathogenesis, we explored whether dysregulation hindlimb reflects phenotypic difference between two mouse models.Ex vivo immunohistochemical, biochemical,...
ABSTRACT Introduction : RNS60 is a novel immune‐modulatory agent that has shown neuroprotective effects in amytrophic lateral sclerosis (ALS) preclinical models. administered by weekly intravenous infusion and daily nebulization. The objective of this pilot open‐label trial was to test the feasibility, safety, tolerability long‐term administration ALS patients. Methods planned treatment duration 23 weeks primary outcomes were safety tolerability. Secondary included PBR28 positron emission...
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with no recognized clinical prognostic factor. Creatinine kinase (CK) increase in these patients already described conflicting results on prognosis and survival. In 126 ALS who were fast or slow progressors, CK levels assayed for 16 months every 4 an observational case-control cohort study prospective data collection conducted Italy. was also measured at baseline 88 CIDP secondary axonal damage two mouse strains (129SvHSD...
Abstract Loss-of-function mutations in the ribonuclease angiogenin are associated with amyotrophic lateral sclerosis. Angiogenin has been shown to cleave transfer RNAs during stress produce ‘transfer-derived stress-induced RNAs’. Stress-induced tRNA cleavage is preserved from single-celled organisms humans indicating it represents part of a highly conserved response. However, date, role sclerosis remains be fully elucidated. To this end, we performed small RNA sequencing on human astrocytoma...
Monocyte chemoattractant protein-1 (MCP1) is one of the most powerful pro-inflammatory chemokines. However, its signaling pivotal in driving injured axon and muscle regeneration. We previously reported that MCP1 more strongly upregulated nervous system slow-progressing than fast-progressing SOD1G93A mice, latter showing a poor immune response eventual massive nerve degeneration. To assess MCP1-mediated therapeutic role, we boosted chemokine along motor unit two models through single...
Amyotrophic Lateral Sclerosis (ALS) is a devastating neurodegenerative disorder characterized by the progressive loss of motor neurons, with genetic and environmental factors contributing to its complex pathogenesis. Dysregulated immune responses altered energetic metabolism are key features, emerging evidence implicating gut microbiota (GM) in disease progression. We investigated interplay among background, GM composition, metabolism, response two distinct ALS mouse models: 129Sv_G93A...
CXCL13 is a B and T lymphocyte chemokine that mediates neuroinflammation through its receptor CXCR5. This highly expressed by motoneurons (MNs) in Amyotrophic Lateral Sclerosis (ALS) SOD1G93A (mSOD1) mice during the disease, particularly fast-progressing mice. Accordingly, this study, we investigated role of ALS.
Muscle wasting occurs during various chronic diseases and precedes death in humans as mice. The evaluation of the degree muscle atrophy diseased mouse models is often overlooked since it requires sacrifice animals for examination or expensive instrumentation highly qualified personnel, such Magnetic Resonance Imaging (MRI). Very behavioral tests strength are used an outcome measurement preclinical therapeutic trials. However, these easy to perform serially, but not enough sensitive detect...
Amyotrophic Lateral Sclerosis (ALS) is a neural disorder gradually leading to paralysis of the whole body. Alterations in superoxide dismutase SOD1 gene have been linked with several variants familial ALS. Here, we investigated transgenic (Tg) cloned swine model expressing human pathological hSOD1G93A allele. As patients, these Tg pigs transmitted disease progeny an autosomal dominant trait and showed ALS onset from about 27 months age. Post mortem analysis revealed motor neuron (MN)...
Abstract Microglial activation plays central roles in neuro-inflammatory and neurodegenerative diseases. Positron emission tomography (PET) targeting 18kDa Translocator Protein (TSPO) is widely used for localising inflammation vivo , but its quantitative interpretation remains uncertain. We show that TSPO expression increases activated microglia mouse brain disease models does not change a non-human primate model or common neuroinflammatory human describe genetic divergence the gene...
Abstract Microglial activation plays central roles in neuro-inflammatory and neurodegenerative diseases. Positron emission tomography (PET) targeting 18kDa Translocator Protein (TSPO) is widely used for localising inflammation vivo, but its quantitative interpretation remains uncertain. For the first time, we show that TSPO gene protein expression increases activated microglia mouse brain disease models postmortem, does not change a non-human primate (Callithrix jacchus) model or common...
<title>Abstract</title> <bold><underline>Background:</underline></bold> Monocyte chemoattractant protein 1 (MCP1/CCL2) is one of the most powerful pro-inflammatory chemokines. However, its signalling pivotal in driving axonal and muscle regeneration following injury. We previously showed that MCP1 strongly upregulated nervous system slow-progressing than fast-progressing SOD1<sup>G93A</sup> mice, which are characterised by a poor immune response leads to massive nerve...