A5024665171- Hereditary Neurological Disorders
- Endoplasmic Reticulum Stress and Disease
- RNA regulation and disease
- Nerve injury and regeneration
- Lysosomal Storage Disorders Research
- Trigeminal Neuralgia and Treatments
- Multiple Sclerosis Research Studies
- Migraine and Headache Studies
- Cellular Mechanics and Interactions
- Trypanosoma species research and implications
- Fibromyalgia and Chronic Fatigue Syndrome Research
- Alzheimer's disease research and treatments
- Neuroblastoma Research and Treatments
- Cellular transport and secretion
- Facial Nerve Paralysis Treatment and Research
- Cerebral Venous Sinus Thrombosis
- Long-Term Effects of COVID-19
- Retinal Development and Disorders
- Prion Diseases and Protein Misfolding
- Genetic Neurodegenerative Diseases
- Amyotrophic Lateral Sclerosis Research
- Autism Spectrum Disorder Research
- Botulinum Toxin and Related Neurological Disorders
- Tendon Structure and Treatment
- Protease and Inhibitor Mechanisms
IRCCS Istituto Auxologico Italiano
2019-2025
Istituti di Ricovero e Cura a Carattere Scientifico
2025
San Raffaele University of Rome
2009-2016
Center for Neurosciences
2016
Vita-Salute San Raffaele University
2011-2013
IRCCS Ospedale San Raffaele
2013
P0 glycoprotein is an abundant product of terminal differentiation in myelinating Schwann cells. The mutant P0S63del causes Charcot-Marie-Tooth 1B neuropathy humans, and a very similar demyelinating transgenic mice. retained the endoplasmic reticulum cells, where it promotes unfolded protein stress elicits response (UPR) associated with translational attenuation. Ablation Chop, UPR mediator, from S63del mice completely rescues their motor deficit reduces active demyelination by half. Here,...
Headache is very often the cause for seeking an emergency department (ED). However, less known about different diagnosis of headache disorders in ED, their management and treatment. The aim this survey to analyse patients two ED Europe.This retrospective was performed from September 2018 until January 2019. Patients were collected San Luca Hospital, Milan, Italy Ordensklinikum Barmherzige Schwestern, Linz, Austria. Only with a non-traumatic headache, as primary reason medical clarification,...
Axonal sorting is a crucial event in nerve formation and requires proper Schwann cell proliferation, differentiation, contact with axons. Any defect axonal results dysmyelinating peripheral neuropathies. Evidence from mouse models shows that regulated by laminin211- and, possibly, neuregulin 1 (Nrg1)-derived signals. However, how these signals are integrated cells largely unknown. We now report the nuclear Jun activation domain-binding protein (Jab1) may transduce laminin211 to regulate...
In factory cells, the accumulation of misfolded protein provokes unfolded response (UPR). For example, deletion serine 63 (S63del) in myelin zero (P0) induces P0 endoplasmic reticulum (ER) Schwann cells and a persistent UPR associated with Charcot-Marie-Tooth 1B (CMT1B) demyelinating peripheral neuropathy human mouse. PERK (protein kinase RNA-like ER kinase) is stress sensor that attenuates global translation by phosphorylating eIF2α. Inhibition eIF2α holophosphatase GADD34:PP1, increases...
In peripheral nerves, P0 glycoprotein accounts for more than 20% of myelin protein content. is synthesized by Schwann cells, processed in the endoplasmic reticulum (ER) and enters secretory pathway. However, mutant with S63 deleted (P0S63del) accumulates ER lumen induces a demyelinating neuropathy Charcot–Marie–Tooth disease type 1B (CMT1B)–S63del mice. Accumulation P0S63del triggers persistent unfolded response. Protein kinase RNA-like (PERK) an stress sensor that phosphorylates eukaryotic...
To identify clinical predictors of effectiveness a motor rehabilitation treatment in cohort multiple sclerosis (MS) patients.We analysed 212 consecutive patients who underwent short-term (3-7 weeks) intensive (two hours per day, five days week), individualised, goal-oriented inpatient program. Activity limitation and impairment were measured on admission discharge the trial using sub-items Functional Independence Measure (mFIM) Expanded Disability Status Scale (EDSS) score. Multivariate...
Remodeling of extracellular matrix (ECM) is a critical step in peripheral nerve regeneration. In fact, human neuropathies, endoneurial ECM enriched fibrin and vitronectin associates with poor regeneration worse clinical prognosis. Accordingly animal models, modification the fibrinolytic complex activity has profound effects on regeneration: high low levels correlate better The urokinase plasminogen receptor (uPAR) major component complex, binding to activator (uPA) promotes fibrinolysis cell...
Growing evidence indicates that alterations within the peripheral nervous system (PNS) are involved at an early stage in amyotrophic lateral sclerosis (ALS) pathogenetic cascade. In this study, magnetic resonance imaging (MRI), neurophysiologic analyses, and histologic analyses were used to monitor extent of PNS damage hSOD-1 ALS rat model. The signature disease was defined using vivo MRI sciatic nerve. Initial abnormalities detected nerves by increase T2 relaxation time before onset...