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New Zealand Veterinary Journal Volume 50, 2002 - Issue 5
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Clinical Communication

Neuronal ceroid-lipofuscinosis in Borderdale sheep

RD Jolly Institute of Veterinary, Animal and Biomedical Sciences, Massey University, Palmerston North, New Zealand E-mail: [email protected]
,
DG Arthur Selwyn Rakaia Vet Services, PO Box 52, Dunsandel 8190, New Zealand
,
GW Kay Animal and Food Science Division, PO Box 84, Lincoln University, Canterbury, New Zealand
&
DN Palmer Animal and Food Science Division, PO Box 84, Lincoln University, Canterbury, New Zealand
Pages 199-202 | Accepted 28 Jun 2002, Published online: 22 Feb 2011

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RD Jolly, KE Dittmer, BR Jones, AJ Worth, KG Thompson, AC Johnstone, DN Palmer, NS Van de Water, KM Hemsley, DJ Garrick, BG Winchester & SU Walkley. (2021) Animal medical genetics: a historical perspective on more than 50 years of research into genetic disorders of animals at Massey University. New Zealand Veterinary Journal 69:5, pages 255-266.
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Jonathan B. Rosenberg, Alvin Chen, Stephen M. Kaminsky, Ronald G. Crystal & Dolan Sondhi. (2019) Advances in the treatment of neuronal ceroid lipofuscinosis. Expert Opinion on Orphan Drugs 7:11, pages 473-500.
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RD Jolly, HT Blair & AC Johnstone. (2004) Genetic disorders of sheep in New Zealand: A review and perspective. New Zealand Veterinary Journal 52:2, pages 52-64.
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Articles from other publishers (31)

Samantha J. Murray, Martin P. Wellby, Graham K. Barrell, Katharina N. Russell, Ashley R. Deane, John R. Wynyard, Steven J. Gray, David N. Palmer & Nadia L. Mitchell. (2023) Efficacy of dual intracerebroventricular and intravitreal CLN5 gene therapy in sheep prompts the first clinical trial to treat CLN5 Batten disease. Frontiers in Pharmacology 14.
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Nadia L. Mitchell, Samantha J. Murray, Martin P. Wellby, Graham K. Barrell, Katharina N. Russell, Ashley R. Deane, John R. Wynyard, Madeleine J. Palmer, Anila Pulickan, Phillipa M. Prendergast, Widler Casy, Steven J. Gray & David N. Palmer. (2023) Long-term safety and dose escalation of intracerebroventricular CLN5 gene therapy in sheep supports clinical translation for CLN5 Batten disease. Frontiers in Genetics 14.
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Nadia L. Mitchell, Katharina N. Russell, Graham K. Barrell, Imke Tammen & David N. Palmer. (2023) Characterization of neuropathology in ovine CLN5 and CLN6 neuronal ceroid lipofuscinoses (Batten disease). Developmental Neurobiology.
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Samantha J Murray, Mustafa M Almuqbel, Simon A Felton, Nickolas J Palmer, Daniel J Myall, Reza Shoorangiz, Arsène Ella, Matthieu Keller, David N Palmer, Tracy R Melzer & Nadia L Mitchell. (2023) Progressive MRI brain volume changes in ovine models of CLN5 and CLN6 neuronal ceroid lipofuscinosis. Brain Communications 5:1.
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I. G. Joe Mayhew & Robert J. MacKay. 2022. Large Animal Neurology. Large Animal Neurology 215 291 .
Hayley Hunt, Keren E. Dittmer, Dorian J. Garrick, Robert A. Fairley, Stephen J. Heap & Robert D. Jolly. (2022) An inherited night blindness in Wiltshire sheep. Veterinary Pathology 59:2, pages 310-318.
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Samantha J. Murray & Nadia L. Mitchell. (2022) The Translational Benefits of Sheep as Large Animal Models of Human Neurological Disorders. Frontiers in Veterinary Science 9.
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Natasha Elizabeth Mckean, Renee Robyn Handley & Russell Grant Snell. (2021) A Review of the Current Mammalian Models of Alzheimer’s Disease and Challenges That Need to Be Overcome. International Journal of Molecular Sciences 22:23, pages 13168.
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Samantha J. Murray, Katharina N. Russell, Tracy R. Melzer, Steven J. Gray, Stephen J. Heap, David N. Palmer & Nadia L. Mitchell. (2021) Intravitreal gene therapy protects against retinal dysfunction and degeneration in sheep with CLN5 Batten disease. Experimental Eye Research 207, pages 108600.
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I. Basak, H. E. Wicky, K. O. McDonald, J. B. Xu, J. E. Palmer, H. L. Best, S. Lefrancois, S. Y. Lee, L. Schoderboeck & S. M. Hughes. (2021) A lysosomal enigma CLN5 and its significance in understanding neuronal ceroid lipofuscinosis. Cellular and Molecular Life Sciences 78:10, pages 4735-4763.
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Hamdi AVCI, Sümbül Serap BİRİNCİOĞLU, Erkmen Tuğrul EPİKMEN & Ahmet AYDOĞAN. (2021) Neuronal Lipofuscinosis in a Hairy GoatBir Kıl Keçisinde Nöronal Lipofusinozis. Erciyes Üniversitesi Veteriner Fakültesi Dergisi 18:1, pages 62-64.
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Wenfei Liu, Sophia-Martha kleine-Holthaus, Saul Herranz-Martin, Mikel Aristorena, Sara E. Mole, Alexander J. Smith, Robin R. Ali & Ahad A. Rahim. (2020) Experimental gene therapies for the NCLs. Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease 1866:9, pages 165772.
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Robert J. Huber, Stephanie M. Hughes, Wenfei Liu, Alan Morgan, Richard I. Tuxworth & Claire Russell. (2020) The contribution of multicellular model organisms to neuronal ceroid lipofuscinosis research. Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease 1866:9, pages 165614.
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Valerjans Kauss, Maija Dambrova & Diego Luis Medina. (2020) Pharmacological approaches to tackle NCLs. Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease 1866:9, pages 165553.
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Nadia L. Mitchell, Katharina N. Russell, Martin P. Wellby, Hollie E. Wicky, Lucia Schoderboeck, Graham K. Barrell, Tracy R. Melzer, Steven J. Gray, Stephanie M. Hughes & David N. Palmer. (2018) Longitudinal In Vivo Monitoring of the CNS Demonstrates the Efficacy of Gene Therapy in a Sheep Model of CLN5 Batten Disease. Molecular Therapy 26:10, pages 2366-2378.
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Katharina N. Russell, Nadia L. Mitchell, Nigel G. Anderson, Craig R. Bunt, Martin P. Wellby, Tracy R. Melzer, Graham K. Barrell & David N. Palmer. (2018) Computed tomography provides enhanced techniques for longitudinal monitoring of progressive intracranial volume loss associated with regional neurodegeneration in ovine neuronal ceroid lipofuscinoses. Brain and Behavior 8:9, pages e01096.
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Hannah L Best, Nicole J Neverman, Hollie E Wicky, Nadia L Mitchell, Beulah Leitch & Stephanie M Hughes. (2017) Characterisation of early changes in ovine CLN5 and CLN6 Batten disease neural cultures for the rapid screening of therapeutics. Neurobiology of Disease 100, pages 62-74.
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Greg M. Cronin, Danai F. Beganovic, Amanda L. Sutton, David J. Palmer, Peter C. Thomson & Imke Tammen. (2016) Manifestation of neuronal ceroid lipofuscinosis in Australian Merino sheep: Observations on altered behaviour and growth. Applied Animal Behaviour Science 175, pages 32-40.
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Inês S. Amorim, Nadia L. Mitchell, David N. Palmer, Stephen J. Sawiak, Roger Mason, Thomas M. Wishart & Thomas H. Gillingwater. (2015) Molecular neuropathology of the synapse in sheep with CLN 5 Batten disease . Brain and Behavior 5:11.
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David N. Palmer, Nicole J. Neverman, Jarol Z. Chen, Chia-Tien Chang, Peter J. Houweling, Lucy A. Barry, Imke Tammen, Stephanie M. Hughes & Nadia L. Mitchell. (2015) Recent studies of ovine neuronal ceroid lipofuscinoses from BARN, the Batten Animal Research Network. Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease 1852:10, pages 2279-2286.
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Kiterie M.E. Faller, Rodrigo Gutierrez-Quintana, Alamin Mohammed, Ahad A. Rahim, Richard I. Tuxworth, Kim Wager & Michael Bond. (2015) The neuronal ceroid lipofuscinoses: Opportunities from model systems. Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease 1852:10, pages 2267-2278.
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Thomas Furmston, A. Jennifer Morton & Stephen Hailes. (2015) A Significance Test for Inferring Affiliation Networks from Spatio-Temporal Data. PLOS ONE 10:7, pages e0132417.
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Nicholas Perentos, Amadeu Q. Martins, Thomas C. Watson, Ullrich Bartsch, Nadia L. Mitchell, David N. Palmer, Matthew W. Jones & A. Jennifer Morton. (2015) Translational neurophysiology in sheep: measuring sleep and neurological dysfunction in CLN5 Batten disease affected sheep. Brain 138:4, pages 862-874.
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Stephanie M. Hughes, Katie M. Hope, Janet Boyu Xu, Nadia L. Mitchell & David N. Palmer. (2014) Inhibition of storage pathology in prenatal CLN5-deficient sheep neural cultures by lentiviral gene therapy. Neurobiology of Disease 62, pages 543-550.
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Michael Bond, Sophia-Martha kleine Holthaus, Imke Tammen, Guy Tear & Claire Russell. (2013) Use of model organisms for the study of neuronal ceroid lipofuscinosis. Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease 1832:11, pages 1842-1865.
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Krystal Weber & David A. Pearce. (2013) Large Animal Models for Batten Disease. Journal of Child Neurology 28:9, pages 1123-1127.
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Kathryn S. LintermanDavid N. PalmerGraham W. KayLucy A. BarryNadia L. MitchellRobin G. McFarlaneMichael A. BlackMark S. SandsStephanie M. Hughes. (2011) Lentiviral-Mediated Gene Transfer to the Sheep Brain: Implications for Gene Therapy in Batten Disease. Human Gene Therapy 22:8, pages 1011-1020.
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Tony Frugier, Nadia L. Mitchell, Imke Tammen, Peter J. Houweling, Donald G. Arthur, Graham W. Kay, Otto P. van Diggelen, Robert D. Jolly & David N. Palmer. (2008) A new large animal model of CLN5 neuronal ceroid lipofuscinosis in Borderdale sheep is caused by a nucleotide substitution at a consensus splice site (c.571 + 1G >>> A) leading to excision of exon 3. Neurobiology of Disease 29:2, pages 306-315.
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. 2007. Jubb, Kennedy & Palmer's Pathology of Domestic Animals. Jubb, Kennedy & Palmer's Pathology of Domestic Animals 322 332 .
Imke Tammen, Peter J. Houweling, Tony Frugier, Nadia L. Mitchell, Graham W. Kay, Julie A.L. Cavanagh, Roger W. Cook, Herman W. Raadsma & David N. Palmer. (2006) A missense mutation (c.184C>T) in ovine CLN6 causes neuronal ceroid lipofuscinosis in Merino sheep whereas affected South Hampshire sheep have reduced levels of CLN6 mRNA. Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease 1762:10, pages 898-905.
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Jonathan D. Cooper. (2003) Progress towards understanding the neurobiology of Batten disease or neuronal ceroid lipofuscinosis. Current Opinion in Neurology 16:2, pages 121-128.
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