EMANUELA D'ACUNTO

Dottoressa di ricerca

ciclo: XXXII



Titolo della tesi: Serpin function and disease: cellular toxicity of neuroserpin polymers and novel roles of alpha1-antitrypsin

The serpinopathies are due to misfolding and intracellular polymerisation of mutant serpin variants within the endoplasmic reticulum of the cell of synthesis and can be classified as conformational diseases, which arise when proteins undergo self-association and tissue deposition (Carrell and Lomas, 1997). Examples of human mutant serpins accumulating and causing a toxic gain of function are alpha1 antitrypsin variants underlying liver cirrhosis and mutants of neuroserpin causing the dementia familial encephalopathy with neuroserpin inclusion bodies (FENIB) (Roussel et al., 2011). Here we investigate with diverse approaches very different aspects of both pathologies. In the case of FENIB, following previous work in our laboratory in which we demonstrated the presence of oxidative stress in a novel neuronal model for FENIB neurodegeneration (Guadagno et al., 2017), we concentrate on cellular aspects of neuroserpin polymer toxicity, describing alterations in the morphology and function of the mitochondrial network and their interconnection with oxidative stress and changes in the communication between ER and mitochondria. Regarding alpha1 antitrypsin deficiency, we present the development and characterisation of functional monoclonal antibodies to explore the immunomodulatory roles of this protein.

Produzione scientifica

  • 11573/950793 - 2017 - Neuroserpin polymers cause oxidative stress in a neuronal model of the dementia FENIB (01a Articolo in rivista)
    GUADAGNO, NOEMI ANTONELLA; MORICONI, CLAUDIA; LICURSI, VALERIO; D'ACUNTO, EMANUELA; NISI, PAOLA SERENA; CARUCCI, NICOLETTA; DE JACO, ANTONELLA; CACCI, EMANUELE; NEGRI, RODOLFO; LUPO, GIUSEPPE; MIRANDA BANOS, MARIA ELENA
  • 11573/1380498 - 2020 - Serpin function and disease: cellular toxicity of neuroserpin polymers and novel roles of alpha1-antitrypsin (07a Tesi di Dottorato)
    D'ACUNTO, EMANUELA
  • 11573/1566754 - 2021 - Neuroserpin: structure, function, physiology and pathology (01g Articolo di rassegna (Review))
    D'ACUNTO, EMANUELA; MIRANDA BANOS, MARIA ELENA
  • 11573/1175716 - 2018 - Circulating miR-127-3p as a Potential Biomarker for Differential Diagnosis in Frontotemporal Dementia (01g Articolo di rassegna (Review))
    D'ACUNTO, EMANUELA; TALARICO, GIUSEPPINA; GASPARINI, MARINA; BRUNO, GIUSEPPE
  • 11573/1552616 - 2021 - G392E neuroserpin causing the dementia FENIB is secreted from cells but is not synaptotoxic (01a Articolo in rivista)
    D'ACUNTO, EMANUELA; MIRANDA BANOS, MARIA ELENA
  • 11573/1552606 - 2021 - The Importance of N186 in the Alpha-1-Antitrypsin Shutter Region Is Revealed by the Novel Bologna Deficiency Variant (01a Articolo in rivista)
    D'ACUNTO, EMANUELA; MIRANDA BANOS, MARIA ELENA
  • 11573/1651297 - 2022 - Polymerogenic neuroserpin causes mitochondrial alterations and activates NFκB but not the UPR in a neuronal model of neurodegeneration FENIB (01a Articolo in rivista)
    D'ACUNTO, EMANUELA; GIANFRANCESCO, LORITA; SERANGELI, ILARIA; D'ORSI, MARCO; SABATO, VALENTINA; GUADAGNO, NOEMI ANTONELLA; CARISTI, SILVANA; DE JACO, ANTONELLA; CACCI, EMANUELE; LUPO, GIUSEPPE; MIRANDA BANOS, MARIA ELENA
  • 11573/1172708 - 2018 - Cellular models for the serpinopathies (02a Capitolo o Articolo)
    D'ACUNTO, EMANUELA; MIRANDA BANOS, MARIA ELENA

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