title: Recent studies of ovine neuronal ceroid lipofuscinoses from BARN, the Batten Animal Research Network.
abstract: 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)".
authors.affiliation: Molecular Biosciences, Faculty of Agriculture and Life Sciences, PO Box 85084, Lincoln University, Lincoln 7647, New Zealand; Batten Animal Research Network (BARN) (www.BARN.org.nz). Electronic address: david.palmer@lincoln.ac.nz.
authors.forename: David N
authors.initials: DN
authors.lastname: Palmer
authors.affiliation: Department of Biochemistry, Otago School of Medical Sciences, Brain Health Research Centre, University of Otago, PO Box 56, Dunedin 9054, New Zealand; Batten Animal Research Network (BARN) (www.BARN.org.nz).
authors.forename: Nicole J
authors.initials: NJ
authors.lastname: Neverman
authors.affiliation: Molecular Biosciences, Faculty of Agriculture and Life Sciences, PO Box 85084, Lincoln University, Lincoln 7647, New Zealand; Batten Animal Research Network (BARN) (www.BARN.org.nz).
authors.forename: Jarol Z
authors.initials: JZ
authors.lastname: Chen
authors.affiliation: Faculty of Veterinary Science, University of Sydney, PMB 4003, Narellan, NSW 2567, Australia; Batten Animal Research Network (BARN) (www.BARN.org.nz).
authors.forename: Chia-Tien
authors.initials: CT
authors.lastname: Chang
authors.affiliation: Murdoch Childrens Research Institute, Royal Children's Hospital, Flemington Road, Parkville, Melbourne, Victoria 3052, Australia; Batten Animal Research Network (BARN) (www.BARN.org.nz).
authors.forename: Peter J
authors.initials: PJ
authors.lastname: Houweling
authors.affiliation: Molecular Biosciences, Faculty of Agriculture and Life Sciences, PO Box 85084, Lincoln University, Lincoln 7647, New Zealand; Batten Animal Research Network (BARN) (www.BARN.org.nz).
authors.forename: Lucy A
authors.initials: LA
authors.lastname: Barry
authors.affiliation: Faculty of Veterinary Science, University of Sydney, PMB 4003, Narellan, NSW 2567, Australia; Batten Animal Research Network (BARN) (www.BARN.org.nz).
authors.forename: Imke
authors.initials: I
authors.lastname: Tammen
authors.affiliation: Department of Biochemistry, Otago School of Medical Sciences, Brain Health Research Centre, University of Otago, PO Box 56, Dunedin 9054, New Zealand; Batten Animal Research Network (BARN) (www.BARN.org.nz).
authors.forename: Stephanie M
authors.initials: SM
authors.lastname: Hughes
authors.affiliation: Molecular Biosciences, Faculty of Agriculture and Life Sciences, PO Box 85084, Lincoln University, Lincoln 7647, New Zealand; Batten Animal Research Network (BARN) (www.BARN.org.nz).
authors.forename: Nadia L
authors.initials: NL
authors.lastname: Mitchell
keywords: Animal models; Batten Disease; CLN5; CLN6; Disease associated cardiology; Gene therapy; NCLs; Neural cultures; Neurodegeneration; Neuroinflammation; Neuroinflammatory pathway; Neuronal ceroid lipofuscinoses