Thanks to its significant evolutionary conservation of eukaryotic biochemical pathways, the yeast Saccharomyces cerevisiae has been employed as a model organism in order to study and unravel the basis of a wide variety of human cellular processes, such as neuronal proteotoxic stress and its implication in cell ageing and death [1]. Thus, our purpose is to use yeast as a model to study a neurodegenerative autosomal dominant disorder called FENIB (Familial Encephalopathy with Neuroserpin Inclusion Bodies). FENIB is a rare conformational disease caused by the aberrant folding of neuroserpin: different mutations in its coding sequence (NS1) induce the polymerization of the protein and its subsequent intracellular deposition, which promotes proteotoxic stress. Severity and age of onset of the symptoms – such as dementia, epilepsy, convulsions and tremor - depend on the specific mutation. Even though different cellular and organism models have been used, no cure has been discovered yet [2]: for this reason, our research group is working on the development and implementation of S. cereviasiae as a new FENIB cellular model, therefore validating yeast as an efficient tool to study neurodegenerative disorders. Through the identification of the molecular mechanisms and the pathways involved, a humanized yeast model could allow a better understanding of the disease onset, progression and cytotoxicity-induced, finding out new possible therapeutic targets. References: [1] Fruhmann, G et al.,. (2017) Yeast buddies helping to unravel the complexity of neurodegenerative disorders. Mech Ageing Dev. Jan;161(Pt B):288-305. [2] Guadagno, NA et al., (2017) Neuroserpin polymers cause oxidative stress in a neuronal model of the dementia FENIB. Neurobiology of Disease, 103:32-44

Development of a yeast model to investigate the molecular aspects involved in the neurodegenerative disease FENIB / Vapore, Valentina; Stirpe, Mariarita; Rinaldi, Teresa; Cirigliano, Angela; MIRANDA BANOS, MARIA ELENA; Mazzaglia, Corrado; Sibilia, Diego; Mazzoni, Cristina. - (2018). ((Intervento presentato al convegno From Stress Response to Tissue Development and Regeneration 28-29 September 2018 tenutosi a Pavia.

Development of a yeast model to investigate the molecular aspects involved in the neurodegenerative disease FENIB

Teresa Rinaldi;Maria Elena Miranda Banos;Cristina Mazzoni
2018

Abstract

Thanks to its significant evolutionary conservation of eukaryotic biochemical pathways, the yeast Saccharomyces cerevisiae has been employed as a model organism in order to study and unravel the basis of a wide variety of human cellular processes, such as neuronal proteotoxic stress and its implication in cell ageing and death [1]. Thus, our purpose is to use yeast as a model to study a neurodegenerative autosomal dominant disorder called FENIB (Familial Encephalopathy with Neuroserpin Inclusion Bodies). FENIB is a rare conformational disease caused by the aberrant folding of neuroserpin: different mutations in its coding sequence (NS1) induce the polymerization of the protein and its subsequent intracellular deposition, which promotes proteotoxic stress. Severity and age of onset of the symptoms – such as dementia, epilepsy, convulsions and tremor - depend on the specific mutation. Even though different cellular and organism models have been used, no cure has been discovered yet [2]: for this reason, our research group is working on the development and implementation of S. cereviasiae as a new FENIB cellular model, therefore validating yeast as an efficient tool to study neurodegenerative disorders. Through the identification of the molecular mechanisms and the pathways involved, a humanized yeast model could allow a better understanding of the disease onset, progression and cytotoxicity-induced, finding out new possible therapeutic targets. References: [1] Fruhmann, G et al.,. (2017) Yeast buddies helping to unravel the complexity of neurodegenerative disorders. Mech Ageing Dev. Jan;161(Pt B):288-305. [2] Guadagno, NA et al., (2017) Neuroserpin polymers cause oxidative stress in a neuronal model of the dementia FENIB. Neurobiology of Disease, 103:32-44
From Stress Response to Tissue Development and Regeneration 28-29 September 2018
04 Pubblicazione in atti di convegno::04d Abstract in atti di convegno
Development of a yeast model to investigate the molecular aspects involved in the neurodegenerative disease FENIB / Vapore, Valentina; Stirpe, Mariarita; Rinaldi, Teresa; Cirigliano, Angela; MIRANDA BANOS, MARIA ELENA; Mazzaglia, Corrado; Sibilia, Diego; Mazzoni, Cristina. - (2018). ((Intervento presentato al convegno From Stress Response to Tissue Development and Regeneration 28-29 September 2018 tenutosi a Pavia.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1610069
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