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Showing 1 to 8 of 8 for “"Settore MEDS-12/A - Neurologia"”.

  1. EXPLORING MITOCHONDRIAL AND REDOX VULNERABILITY IN FRIEDREICH'S ATAXIA USING GOLD NANOCLUSTERS IN HUMAN CELLULAR MODELS

    Friedreich’s ataxia (FRDA) is a rare autosomal recessive neurodegenerative disorder caused by transcriptional silencing of the FXN gene, leading to frataxin deficiency, impaired iron–sulfur cluster biogenesis, mitochondrial dysfunction, and chronic oxidative stress. Despite significant advances in …

    milano Repository record for EXPLORING MITOCHONDRIAL AND REDOX VULNERABILITY IN FRIEDREICH'S ATAXIA USING GOLD NANOCLUSTERS IN HUMAN CELLULAR MODELS (opens in a new tab)

  2. PATHOGENETIC DEFINITION OF THE MECHANISMS UNDERLYING THE NOVEL FAMILY OF PLIN4 GENE EXPANSION-RELATED MYOPATHY

    … motivo ho iniziato a lavorare con cellule C2C12, una linea di mioblasti derivati da topo, capaci eventualmente di ricapitolare in maniera più accurata le caratteristiche muscolari. Due linee stabili in cellule C2C12 sono quindi state generate e la validazione di questi modelli è attualmente …

    milano Repository record for PATHOGENETIC DEFINITION OF THE MECHANISMS UNDERLYING THE NOVEL FAMILY OF PLIN4 GENE EXPANSION-RELATED MYOPATHY (opens in a new tab)

  3. ACUTE ISCHEMIC STROKE: CANCER-RELATED STROKE AND MULTIMODAL ANALYSIS OF THROMBI.

    Background Acute ischemic stroke (AIS) usually results from atherosclerosis or cardiac sources, but less frequent mechanisms, such as cancer-related stroke, play a role in some cases. Studying these rarer causes of AIS is crucial for secondary prevention. Thrombi analysis provides a unique window …

    milano Repository record for ACUTE ISCHEMIC STROKE: CANCER-RELATED STROKE AND MULTIMODAL ANALYSIS OF THROMBI. (opens in a new tab)

  4. CARDIAC AUTONOMIC CONTROL DURING SLEEP IN PATIENTS WITH MULTIPLE SYSTEM ATROPHY: A PILOT STUDY ON THE IMPACT OF CPAP THERAPY

    Objectives To characterize sleep-stage autonomic regulation in multiple system atrophy (MSA) using cardiorespiratory coupling (K2) and symbolic dynamics (0V%, 2UV%, 2LV%) derived from ECG tracings in video‐polysomnography (v‐PSG). The study aims to compare patients with and without sleep‐disordered …

    milano Repository record for CARDIAC AUTONOMIC CONTROL DURING SLEEP IN PATIENTS WITH MULTIPLE SYSTEM ATROPHY: A PILOT STUDY ON THE IMPACT OF CPAP THERAPY (opens in a new tab)

  5. DTI-ALPS REFLECTS WHITE MATTER DIFFUSION ANISOTROPY AND MICROSTRUCTURAL HETEROGENEITY RATHER THAN GLYMPHATIC FLOW: INSIGHTS FROM MULTI-MODAL MRI

    The glymphatic system plays a critical role in the clearance of metabolic waste and pathological proteins such as amyloid-β and tau from the brain, and its dysfunction has been increasingly implicated in the pathogenesis of neurodegenerative diseases, making its in vivo characterization a major …

    milano Repository record for DTI-ALPS REFLECTS WHITE MATTER DIFFUSION ANISOTROPY AND MICROSTRUCTURAL HETEROGENEITY RATHER THAN GLYMPHATIC FLOW: INSIGHTS FROM MULTI-MODAL MRI (opens in a new tab)

  6. CARDIOVASCULAR AUTONOMIC IMPAIRMENT IN GBA PARKINSONIAN PATIENTS

    … supine and standing positions. Additionally, 123I-MIBG myocardial single-photon emission CT was used to evaluate postganglionic sympathetic nerve terminals. Results: A total of 34 GBA carriers and 33 I-PD patients were included. GBA-PD patients exhibited greater severity of autonomic symptoms …

    milano Repository record for CARDIOVASCULAR AUTONOMIC IMPAIRMENT IN GBA PARKINSONIAN PATIENTS (opens in a new tab)

  7. MODELLING RIBOFLAVIN TRANSPORTER DEFICIENCY (RTD) USING IPSC-DERIVED MODELS TO TEST GENE THERAPY EFFICACY

    Riboflavin transporter deficiency syndrome (RTD) is a rare childhood-onset neurodegenerative disorder caused by mutations in SLC52A2 and SLC52A3 genes, encoding the riboflavin (RF) transporters RFVT2 and RFVT3. In the present study we focused on RTD Type 2, which is due to variants in SLC52A2 gene. …

    milano Repository record for MODELLING RIBOFLAVIN TRANSPORTER DEFICIENCY (RTD) USING IPSC-DERIVED MODELS TO TEST GENE THERAPY EFFICACY (opens in a new tab)

  8. SMN DEFICIENCY INDUCES NEUROACTIVE AMINOACIDS DYSMETABOLISM: BRIDGING INSIGHTS FROM PRECLINICAL MODELS TO CLINICAL EVIDENCE IN SMA PATIENTS

    ABSTRACT Spinal muscular atrophy (SMA) is a leading genetic cause of infant mortality, driven by homozygous loss of the SMN1 gene and reduced SMN protein. Although disease-modifying therapies such as Nusinersen, Risdiplam, and Onasemnogene abeparvovec have markedly improved survival and motor …

    milano Repository record for SMN DEFICIENCY INDUCES NEUROACTIVE AMINOACIDS DYSMETABOLISM: BRIDGING INSIGHTS FROM PRECLINICAL MODELS TO CLINICAL EVIDENCE IN SMA PATIENTS (opens in a new tab)