Recent studies of ovine neuronal ceroid lipofuscinoses from BARN, the Batten Animal Research Network
| Autor: | Nadia L. Mitchell, Jarol Z. Chen, Peter J. Houweling, David Palmer, Chia-Tien Chang, Lucy A. Barry, Imke Tammen, Nicole J. Neverman, Stephanie M. Hughes |
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| Rok vydání: | 2015 |
| Předmět: |
congenital
hereditary and neonatal diseases and abnormalities Batten disease Brain tissue engineering.material Biology Disease associated cardiology Neural cultures Gene therapy NCLs Neuroinflammation medicine Neurodegeneration Molecular Biology Neuronal Ceroid-Lipofuscinoses CLN5 nutritional and metabolic diseases Batten Disease CLN6 medicine.disease Animal models Batten Differentially expressed genes Neuronal ceroid lipofuscinoses engineering Molecular Medicine WHOLE ANIMAL Neuroscience Neuroinflammatory pathway |
| Zdroj: | Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease. 1852:2279-2286 |
| ISSN: | 0925-4439 |
| Popis: | Studies on naturally occurring New Zealand and Australian ovine models of the neuronal ceroid-lipofuscinoses (Batten disease, NCLs) have greatly aided our understanding of these diseases. Close collaborations between the New Zealand groups at Lincoln University and the University of Otago, Dunedin, and a group at the University of Sydney, Australia, led to the formation of BARN, the Batten Animal Research Network. This review focusses on presentations at the 14th International Conference on Neuronal Ceroid Lipofuscinoses (Batten Disease), recent relevant background work, and previews of work in preparation for publication. Themes include CLN5 and CLN6 neuronal cell culture studies, studies on tissues from affected and control animals and whole animal in vivo studies. Topics include the effect of a CLN6 mutation on endoplasmic reticulum proteins, lysosomal function and the interactions of CLN6 with other lysosomal activities and trafficking, scoping gene-based therapies, a molecular dissection of neuroinflammation, identification of differentially expressed genes in brain tissue, an attempted therapy with an anti-inflammatory drug in vivo and work towards gene therapy in ovine models of the NCLs. This article is part of a Special Issue entitled: "Current Research on the Neuronal Ceroid Lipofuscinoses (Batten Disease)". |
| Databáze: | OpenAIRE |
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