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Dissertations / Theses on the topic 'Neuroserpin'

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Author: Grafiati

Published: 10 December 2022

Last updated: 10 March 2023

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1

Davies, Mark James. "The cytopathology of familial encephalopathy with neuroserpin inclusion bodies (FENIB)." Thesis, University of Cambridge, 2007. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.598334.

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Neuroserpin is a 55 kDa glycoprotein that is secreted from axons of the central and peripheral nervous system. Point mutations within the neuroserpin gene underlie the novel inclusion body dementia Familial Encephalopathy with the Neuroserpin Inclusion Bodies (FENIB). These point mutations destabilise the molecule resulting in the formation of intracellular polymers by sequential insertion of the reactive centre loop of one molecule into β-sheet A of another. Here I postulate that endoplasmic reticulum (ER) inclusions of mutant serpins contribute to the molecular pathogenesis by directing a novel ER stress response. To assess this hypothesis I generated conditional PC-12 Tet-on cell lines expressing wild type neuroserpin, the Ser52Arg and Gly392Glu mutants that underlie FENIB and a novel-misfolding mutant (DeltaNS) predicted to stimulate the unfolded protein response (UPR). The mutants that cause FENIB accumulate within the ER as polymers that I can demonstrate by Western blot analysis and fluorescence confocal microscopy with novel monoclonal antibodies that detect the polymeric conformer of neuroserpin. Despite accumulating, the mutant neuroserpin does not elicit a UPR. However I demonstrate that the ER accumulation of mutant neuroserpin elicits an ER stress response resulting in activation of NF-κB, and this activation is calcium dependent. Taken together, I have used the disease-related neuroserpin inclusions to define and characterise a novel ER derived signalling cascade involved in sensing protein accumulation within the ER.

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2

MacLeod, Ian. "A Drosophila model of familial encephalopathy with neuroserpin inclusion bodies." Thesis, University of Cambridge, 2009. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.611439.

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3

Schipanski, Angela [Verfasser], and Markus [Akademischer Betreuer] Glatzel. "Mechanism of neuronal death in familial encephalopathy with neuroserpin inclusion bodies (FENIB) : Implications for mutant neuroserpin degradation ; a study in mus musculus / Angela Schipanski. Betreuer: Markus Glatzel." Hamburg : Staats- und Universitätsbibliothek Hamburg, 2011. http://d-nb.info/1020458119/34.

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4

Sharp, Lynda Karen. "The biochemical characterisation of mutants of neuroserpin that cause the dementia FENIB." Thesis, University of Cambridge, 2006. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.614202.

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5

Kinghorn, Kerri Jane. "The role of neuroserpin and Aβ in the pathogenesis of Alzheimer's disease." Thesis, University of Cambridge, 2006. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.614058.

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6

Marik, Sergej [Verfasser], and Markus [Akademischer Betreuer] Glatzel. "Mannose-6-Phosphat abhängiger Transport von Neuroserpin / Sergej Marik. Betreuer: Markus Glatzel." Hamburg : Staats- und Universitätsbibliothek Hamburg, 2013. http://d-nb.info/1045024236/34.

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7

Takehara, Sayaka. "Studies on the mechanism for the formation of neuroserpin polymers causing dementia." Kyoto University, 2011. http://hdl.handle.net/2433/142314.

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Kyoto University (京都大学)
0048
新制・課程博士
博士(農学)
甲第16116号
農博第1852号
新制||農||988(附属図書館)
学位論文||H23||N4586(農学部図書室)
28695
京都大学大学院農学研究科応用生命科学専攻
(主査)教授三上文三, 教授植田和光, 教授植田充美
学位規則第4条第1項該当

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8

Lai, Christine Chieh-Lin. "Molecular and cellular studies of Drosophila neuroserpin Spn4A and its polymer-forming mutants." Thesis, University of British Columbia, 2010. http://hdl.handle.net/2429/27742.

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Serpins (Serine Protease Inhibitors) are expressed by most organisms and perform a variety of functions. Most serpins inhibit proteases by undergoing a unique conformational change. They are clinically relevant in two ways. First, introduction of single amino acid point mutations transforms the serpins’ labile conformations into pathogenic, inactive polymers causing “serpinopathies”. In particular, human neuroserpin is a brain-specific serpin that, when mutated, causes a debilitating early onset dementia through unknown cellular pathways. Second, serpins are currently under investigation as therapeutic inhibitors of proprotein convertases (PCs). PCs are associated with some bacterial and viral infections as well as cancer. However, no comprehensive investigation into the cellular effects of PC inhibitor expression in mammalian cells has been performed. This thesis details the use of the Drosophila serpin, Spn4A, to address the cellular pathways mediated by serpin polymers or PC inhibition. Spn4A is a neuron-specific, secretory pathway serpin that inhibits Drosophila or human PCs. We hypothesized that Spn4A mutants, encoding homologous disease-causing mutations in human neuroserpin, would form pathogenic polymers and represent an ideal candidate for generating a cell-based and transgenic Drosophila serpinopathy model. Further, we hypothesized that we could evaluate the cellular response to PC inhibition and polymer accumulation by transcriptome profiling of H4 human neuroglioma cells expressing Spn4A wild-type and mutants. We established an expression system using Spn4A and its mutants in H4s. Subsequently, we used microarray analysis to simultaneously address how serpin polymers may induce cytotoxicity as well as the effects of proprotein processing inhibition in neuroglioma cells. We demonstrated that Spn4A mutants formed polymers, were retained in the endoplasmic reticulum, and lacked inhibitory function, but induced few changes on the transcriptome (under 20 genes differentially regulated). To this end, we have developed transgenic Drosophila overexpressing Spn4A variants to further investigate the biological impact of Spn4A mutants. Next, we analyzed the response to the PC inhibitor, Spn4A, and found marked changes in genes related to malignancy. Our genome-wide gene expression studies have provided novel insights into cellular changes in response to polymeric or PC-inhibiting serpins, and establish the foundation for future functional studies.

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9

Karlsson, Li Susanna Monika. "Structural characterisation of the conformational transitions of neuroserpin that underlie the dementia FENIB." Thesis, University of Cambridge, 2009. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.611426.

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10

Ingwersen, Thies Frieder [Verfasser]. "Using small chemical compounds to inhibit polymerization of neuroserpin in FENIB / Thies Frieder Ingwersen." Hamburg : Staats- und Universitätsbibliothek Hamburg Carl von Ossietzky, 2020. http://d-nb.info/122853778X/34.

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11

Lange, Sascha N. [Verfasser], and Markus [Akademischer Betreuer] Glatzel. "Mechanismus der Neuroserpin-Demenz an einem Caenorhabditis-elegans-Modell (Maupas, 1900) Dougherty, 1953 / Sascha N. Lange. Betreuer: Markus Glatzel." Hamburg : Staats- und Universitätsbibliothek Hamburg, 2011. http://d-nb.info/1020419075/34.

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12

Oberhauser, Felix [Verfasser], and Markus [Akademischer Betreuer] Glatzel. "Beteiligung von OS-9 an der ER-assoziierten Degradation (ERAD) bei familiärer Enzephalopathie mit Neuroserpin-Einschlüssen (FENIB) / Felix Oberhauser. Betreuer: Markus Glatzel." Hamburg : Staats- und Universitätsbibliothek Hamburg, 2014. http://d-nb.info/1055040323/34.

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13

BROGGINI, LUCA. "MOLECULAR DETERMINANTS UNDERLYING PROTEIN MISFOLDING AND AGGREGATION." Doctoral thesis, Università degli Studi di Milano, 2021. http://hdl.handle.net/2434/831967.

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Proteins have evolved to adopt distinctive and well-defined functional states under physiological conditions, either as monomers or complexes. The achievement of a three-dimensional structure allows proteins to exert their physiological functions. Nevertheless, when proteins lose – or fail to acquire – their spatial organization, they can convert into aggregated species that can be harmful to the organism. Conformational diseases gather all those pathologies characterized by the misfolding and aggregation of proteins. Indeed, while the formation and deposition of proteinaceous aggregates can be toxic to cells, the lack of active folded protein disrupts normal physiological pathways. Although considerable progresses have been made in the recent years, to date conformational diseases are still incurable. Indeed, the incomplete understanding of the causes guiding protein misfolding and aggregation prevents the development of efficient treatments. At the same time, the complexity and the diversity of the processes leading to the formation of aggregated species make the task extremely challenging. This PhD project was developed to provide a more comprehensive overview of the molecular bases underlying the conversion of soluble and functional states into aggregated and potentially toxic species. To reach such aims, we applied an integrative approach on two model systems, neuroserpin (NS) and beta-2 microglobulin (2m). In particular, we combined a series of biophysical, biochemical and structural techniques to study these two proteins which have been largely used as model systems for serpin polymerization and amyloid formation, respectively. We found that protein misfolding and aggregation processes depend on several molecular properties, including primary sequence, denatured state compactness, thermal stability, ability to form oligomers under physiological conditions, and the presence of post-translation modifications. The data presented in this PhD thesis add valuable information to depict the complex framework of protein misfolding and aggregation.

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14

Wiesmüller, Felix Johannes Alfred [Verfasser], and Markus [Akademischer Betreuer] Glatzel. "An investigation of interaction between Neuroserpin and its putative targets PC1/3, PC2 and Furin with a YFP-protein complementation assay / Felix Johannes Alfred Wiesmüller. Betreuer: Markus Glatzel." Hamburg : Staats- und Universitätsbibliothek Hamburg, 2016. http://d-nb.info/1084213060/34.

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15

Wiesmüller, Felix Johannes Alfred Verfasser], and Markus [Akademischer Betreuer] [Glatzel. "An investigation of interaction between Neuroserpin and its putative targets PC1/3, PC2 and Furin with a YFP-protein complementation assay / Felix Johannes Alfred Wiesmüller. Betreuer: Markus Glatzel." Hamburg : Staats- und Universitätsbibliothek Hamburg, 2016. http://nbn-resolving.de/urn:nbn:de:gbv:18-77289.

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16

Lebeurrier, Nathalie. "Neuroserpine et ischémie cérébrale / par Nathalie Lebeurrier : rôles et régulation transcriptionnelle." Caen, 2005. http://www.theses.fr/2005CAEN2021.

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Actuellement, le seul traitement de l'ischémie cérébrale, troisième cause de mortalité dans les pays industrialisés, est la thrombolyse par l'activateur tissulaire du plasminogène (tPA). Cependant, bien que bénéfique au niveau vasculaire, le tPA a une action délétère dans le parenchyme cérébral, notamment en modifiant les propriétés du récepteur glutamatergique NMDA, conduisant à la potentialisation de la mort neuronale excitotoxique. Dans cette étude, nous nous sommes intéressés à un inhibiteur endogène du tPA spécifiquement exprimé dans le système nerveux central, la neuroserpine (NS). Dans un premier temps, nous avons montré que la NS réduit la mort neuronale excitotoxique in vitro et in vivo. La NS semble donc avoir un potentiel thérapeutique intéressant dans le cadre de l'ischémie cérébrale, puisqu'elle permettrait de freiner l'activité du tPA dans le parenchyme cérébral sans altérer son action bénéfique au niveau vasculaire. L'injection intraveineuse ou intracérébrale de NS n'étant pas envisageable, nous avons cherché à identifier des agents capables de moduler son expression dans le parenchyme cérébral. Nous avons montré que certains membres de la superfamille du Transforming Growth Factor-beta (TGF-beta) sont capables d'augmenter l'expression de la NS dans les neurones corticaux. Parmi ces membres, nous avons identifié les Bone Morphogenetic Proteins (BMP-2, BMP-4 et BMP-7) et de manière plus surprenante l'hormone anti-Müllerienne (AMH).

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17

D'ACUNTO, EMANUELA. "Serpin function and disease: cellular toxicity of neuroserpin polymers and novel roles of alpha1-antitrypsin." Doctoral thesis, 2020. http://hdl.handle.net/11573/1380498.

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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.

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