{"id":20791,"date":"2024-06-24T16:38:14","date_gmt":"2024-06-24T14:38:14","guid":{"rendered":"https:\/\/ela-international.preprod.melisagency.fr\/?post_type=news_research&p=20791"},"modified":"2024-07-11T11:41:34","modified_gmt":"2024-07-11T09:41:34","slug":"evaluating-the-role-of-the-neuropeptide-n-acetylaspartylglutamate-in-the-pathogenesis-of-canavan-disease-using-new-transgenic-mouse-models","status":"publish","type":"news_research","link":"https:\/\/elainternational.eu\/en\/actualites_recherche\/evaluating-the-role-of-the-neuropeptide-n-acetylaspartylglutamate-in-the-pathogenesis-of-canavan-disease-using-new-transgenic-mouse-models\/","title":{"rendered":"Evaluating the role of the neuropeptide N-acetylaspartylglutamate in the pathogenesis of Canavan disease using new transgenic mouse models"},"content":{"rendered":"\n\n
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Evaluating the role of the neuropeptide N-acetylaspartylglutamate in the pathogenesis of Canavan disease using new transgenic mouse models<\/strong><\/h3>\n <\/div>\n <\/div>\n<\/section>\n\n\n
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Matthias Eckhardt – Institute of Biochemistrv and Molecular Biology, University of Bonn, Bonn, Germany – ELA2021\u2010009I4<\/em><\/p>\n <\/div>\n<\/section>\n\n\n\n\n\n

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Description of the project<\/strong><\/h5>\n <\/div>\n
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Deficiency in the enzyme aspartoacylase leads to a severe leukodystrophy, called Canavan disease, which is characterized by the accumulation of the brain metabolite N-acetylaspartate (NAA). NAA is synthesized and released mainly by neurons. Many neurons use NAA also to synthesize the small neuropeptide N-acetylaspartylglutamate (NAAG). Extracellular NAAG secreted by neurons can be degraded to NAA and glutamate by an enzyme expressed by astrocytes. Thus, degradation of NAAG is another source of NAA. The current project will further investigate the role of NAAG in the NAA accumulation observed in Canavan disease. This will be done using different transgenic mouse models, including a mouse model of Canavan disease, that differ in their capability to synthesize NAAG. In the project, histological and biochemical methods will be used to evaluate the influence of the genetic changes in these mouse models on the accumulation of NAA and the development of brain spongiform degeneration and myelin loss. The aim of this project is to examine whether reduced NAAG synthesis leads to reduced NAA accumulation and reduced pathological changes in the Canavan disease mouse model. If this would be the case, inhibition of NAAG synthesizing enzymes would potentially be an alternative or additional option for a substrate reduction therapy of Canavan disease.<\/p>\n

Project financed by ELA up to: 9 700 \u20ac<\/p>\n <\/div>\n <\/div>\n<\/section>","protected":false},"excerpt":{"rendered":"","protected":false},"author":2,"featured_media":0,"parent":0,"template":"","news_research_categories":[127],"news_research_tags":[120,114],"class_list":["post-20791","news_research","type-news_research","status-publish","hentry","news_research_categories-project","news_research_tags-canavan-disease","news_research_tags-leukodystrophy"],"acf":[],"yoast_head":"\nEvaluating the role of the neuropeptide N-acetylaspartylglutamate in the pathogenesis of Canavan disease using new transgenic mouse models - ELA International<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/elainternational.eu\/en\/actualites_recherche\/evaluating-the-role-of-the-neuropeptide-n-acetylaspartylglutamate-in-the-pathogenesis-of-canavan-disease-using-new-transgenic-mouse-models\/\" \/>\n<meta property=\"og:locale\" content=\"en_US\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Evaluating the role of the neuropeptide N-acetylaspartylglutamate in the pathogenesis of Canavan disease using new transgenic mouse models - 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