A5017229584- Neurogenetic and Muscular Disorders Research
- Muscle Physiology and Disorders
- Liver Disease Diagnosis and Treatment
- Inflammatory Myopathies and Dermatomyositis
- Neuroscience of respiration and sleep
- Inhalation and Respiratory Drug Delivery
- Liver Disease and Transplantation
- Tuberculosis Research and Epidemiology
- Mitochondrial Function and Pathology
- Lysosomal Storage Disorders Research
- Mycobacterium research and diagnosis
- Obstructive Sleep Apnea Research
- Amyotrophic Lateral Sclerosis Research
- Glycogen Storage Diseases and Myoclonus
- Carbohydrate Chemistry and Synthesis
- RNA modifications and cancer
- Adipose Tissue and Metabolism
- Botulinum Toxin and Related Neurological Disorders
- Nuclear Structure and Function
- Muscle and Compartmental Disorders
- Chronic Obstructive Pulmonary Disease (COPD) Research
- Neurological disorders and treatments
- RNA Research and Splicing
- Hepatitis B Virus Studies
- Tracheal and airway disorders
Fondazione IRCCS Istituto Neurologico Carlo Besta
2011-2023
Istituti Clinici Scientifici Maugeri
1996-2019
Istituti di Ricovero e Cura a Carattere Scientifico
2019
Ospedale Valduce
2018
Fondazione Salvatore Maugeri
2003-2016
University of Foggia
2016
University of Milan
2004-2013
University of Pavia
2012
European Institute of Oncology
2012
IRCCS Policlinico San Donato
1974-2008
<b>Background:</b> Transjugular intrahepatic portosystemic shunt (TIPS) is a treatment for portal hypertension-related complications. Accurate prediction of the outcome patients treated with TIPS important, because some have very short survival. Diastolic dysfunction frequently observed in cirrhosis. <b>Aim:</b> To investigate whether or not diastolic can predict after TIPS. <b>Methods:</b> Echocardiography Doppler exploration was performed before and 28 days insertion 32 Several...
Patients with myasthenia gravis (MG) are subgrouped as acetylcholine receptor (AChR)-positive, muscle-specific kinase (MuSK)-positive, and AChR/MuSK-negative MG (or double negative [DN]) on the basis of autoantibody assay. We investigated relationships between specificity, main clinical features, outcome disease, in particular occurrence complete stable remission (CSR), by means a retrospective study cohort 677 Italian patients MG.A total 517 (76%) AChR-positive MG, 55 (8%) MuSK-positive 105...
Autophagy has a large range of physiological functions and its dysregulation contributes to several human disorders, including autoinflammatory/autoimmune diseases such as inflammatory myopathies (IIMs). In order better understand the pathogenetic mechanisms these muscular we sought define role autophagic processes their relation with innate immune system in three main subtypes IIM, specifically sporadic inclusion body myositis (sIBM), polymyositis (PM), dermatomyositis (DM) juvenile (JDM)....
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,...
Parenchymal tissue strips have been used to investigate the mechanical behavior of lung parenchyma. We questioned whether relative amounts alveolar, blood vessel, and bronchial walls would be important when contractile response parenchymal from Sprague-Dawley rats was studied. One group cut subpleural edge another between 1 3 mm proximal pleura. Strips were suspended in an organ bath filled with Krebs solution (37 degrees C, pH 7.4) bubbled 95% O2–5% CO2. Resting tension (T) set at 1.1 g,...
Congenital muscular dystrophies due to defects in genes encoding proteins involved α-dystroglycan (α-DG) glycosylation are a heterogeneous group of muscle disorders variably associated with central nervous system and eye abnormalities. One the more severe is muscle-eye-brain disease (MEB). Mutations coding for proven or putative glycosyltransferases (POMT1, POMT2, POMGnT1, fukutin, FKRP, LARGE), DPM3 gene DOL-P-Man synthase subunit, DAG1 α-dystroglycan, have been altered α-DG...
Laminopathies are a heterogeneous group of diseases, caused by mutations in lamin A/C proteins. The most common laminopathy (LMNA-related myopathies, LMNA-RM) affects skeletal and cardiac muscles; muscle histopathology is variable, ranging from mild unspecific changes to dystrophic features, sometimes with inflammatory evidence. Whether the genetic defect might activate innate immune components, leading chronic inflammation, myofiber necrosis fibrosis, still unknown. By qPCR, significant...
Obstructive sleep apnea syndrome (OSAS) has been shown to be associated upper and lower airways inflammation. Continuous positive airway pressure (CPAP) is the elective treatment of OSAS. The aim present study was assess effect CPAP-therapy on nasal inflammation.In 13 non-smoking subjects affected by untreated OSAS in 11 normal volunteers, inflammation detected analyses induced sputum. In group measurements were repeated after 1, 10 60 days appropriate CPAP treatment. addition, 12 group,...
We aimed to verify whether the immune system may represent a source of potential biomarkers for stratification immune-mediated necrotizing myopathies (IMNMs) subtypes. A group 22 patients diagnosed with IMNM [7 autoantibodies against signal recognition particle (SRP) and 15 3-hydroxy-3-methyl-glutaryl-coenzyme reductase (HMGCR)] 12 controls were included. significant preponderance M1 macrophages was observed in both SRP+ HMGCR+ muscle samples (p < 0.0001 p = 0.0316 ), higher values 0.01)....
Oculopharyngeal muscular dystrophy (OPMD) is a late-onset muscle disease caused by an abnormal (GCN) triplet expansion within the polyadenylate-binding protein nuclear 1 gene and consequent mRNA processing impairment myogenic defects. Because reduced cell proliferation potential regeneration failure of aging have been shown to be governed lethal-7 (let-7) microRNA-mediated mechanisms, in present study, we evaluated role let-7 pathogenesis OPMD. By multidisciplinary approach, including conf...
Large amplitude oscillations of contracted airway smooth muscle cause relative relaxation the preparation. However, little is known about effect mechanical stretch on distal lung behaviour. Rat parenchymal strips were suspended in an organ bath and attached at one end to a force transducer other servo-controlled lever arm that effected length changes. Mechanical impedance strip was measured by applying complex signal consisting pseudorandom varying frequencies (0.5–19.75 Hz). A constant...
Challenges with high concentrations of constrictor agonist delivered by intravenous vs. aerosol result in different modifications the mechanical properties lung tissues. We questioned whether low doses a smooth muscle administered via routes (aerosol, i.v. bolus, continuous infusion) or an increase positive end-expiratory pressure (PEEP) would perturbations Tracheal and alveolar pressures flow were measured open-chest mechanically ventilated (frequency 1 Hz, tidal volume 10 ml/kg, PEEP 4...