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 I Tammen, PJ Houweling, T Frugier, NL Mitchell, GW Kay, JAL Cavanagh, ... Biochimica et Biophysica Acta (BBA)-Molecular Basis of Disease 1762 (10 …, 2006 | 95 | 2006 |
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 … T Frugier, NL Mitchell, I Tammen, PJ Houweling, DG Arthur, GW Kay, ... Neurobiology of disease 29 (2), 306-315, 2008 | 88 | 2008 |
Longitudinal in vivo monitoring of the CNS demonstrates the efficacy of gene therapy in a sheep model of CLN5 Batten disease NL Mitchell, KN Russell, MP Wellby, HE Wicky, L Schoderboeck, ... Molecular Therapy 26 (10), 2366-2378, 2018 | 75 | 2018 |
Neuronal ceroid lipofuscinosis in Devon cattle is caused by a single base duplication (c. 662dupG) in the bovine CLN5 gene PJ Houweling, JAL Cavanagh, DN Palmer, T Frugier, NL Mitchell, ... Biochimica et Biophysica Acta (BBA)-Molecular Basis of Disease 1762 (10 …, 2006 | 62 | 2006 |
Translational neurophysiology in sheep: measuring sleep and neurological dysfunction in CLN5 Batten disease affected sheep N Perentos, AQ Martins, TC Watson, U Bartsch, NL Mitchell, DN Palmer, ... Brain 138 (4), 862-874, 2015 | 55 | 2015 |
Lentiviral-mediated gene transfer to the sheep brain: implications for gene therapy in Batten disease KS Linterman, DN Palmer, GW Kay, LA Barry, NL Mitchell, RG McFarlane, ... Human gene therapy 22 (8), 1011-1020, 2011 | 53 | 2011 |
Inhibition of storage pathology in prenatal CLN5-deficient sheep neural cultures by lentiviral gene therapy SM Hughes, KM Hope, JB Xu, NL Mitchell, DN Palmer Neurobiology of disease 62, 543-550, 2014 | 48 | 2014 |
Recent studies of ovine neuronal ceroid lipofuscinoses from BARN, the Batten Animal Research Network DN Palmer, NJ Neverman, JZ Chen, CT Chang, PJ Houweling, LA Barry, ... Biochimica et Biophysica Acta (BBA)-Molecular Basis of Disease 1852 (10 …, 2015 | 42 | 2015 |
Molecular neuropathology of the synapse in sheep with CLN 5 Batten disease IS Amorim, NL Mitchell, DN Palmer, SJ Sawiak, R Mason, TM Wishart, ... Brain and behavior 5 (11), e00401, 2015 | 41 | 2015 |
Characterisation of early changes in ovine CLN5 and CLN6 Batten disease neural cultures for the rapid screening of therapeutics HL Best, NJ Neverman, HE Wicky, NL Mitchell, B Leitch, SM Hughes Neurobiology of Disease 100, 62-74, 2017 | 40 | 2017 |
An EEG investigation of sleep homeostasis in healthy and CLN5 batten disease affected sheep N Perentos, AQ Martins, RJM Cumming, NL Mitchell, DN Palmer, ... Journal of Neuroscience 36 (31), 8238-8249, 2016 | 37 | 2016 |
The translational benefits of sheep as large animal models of human neurological disorders SJ Murray, NL Mitchell Frontiers in Veterinary Science 9, 831838, 2022 | 35 | 2022 |
Computed tomography provides enhanced techniques for longitudinal monitoring of progressive intracranial volume loss associated with regional neurodegeneration in ovine … KN Russell, NL Mitchell, NG Anderson, CR Bunt, MP Wellby, TR Melzer, ... Brain and behavior 8 (9), e01096, 2018 | 28 | 2018 |
Intravitreal gene therapy protects against retinal dysfunction and degeneration in sheep with CLN5 Batten disease SJ Murray, KN Russell, TR Melzer, SJ Gray, SJ Heap, DN Palmer, ... Experimental Eye Research 207, 108600, 2021 | 26 | 2021 |
Rapid and progressive regional brain atrophy in CLN6 Batten disease affected sheep measured with longitudinal magnetic resonance imaging SJ Sawiak, SR Perumal, SR Rudiger, L Matthews, NL Mitchell, ... PloS one 10 (7), e0132331, 2015 | 25 | 2015 |
The development of brain magnetic resonance approaches in large animal models for preclinical research A Ella, DA Barrière, H Adriaensen, DN Palmer, TR Melzer, NL Mitchell, ... Animal Frontiers 9 (3), 44-51, 2019 | 16 | 2019 |
Rapid detection and characterisation of infectious bronchitis virus (IBV) from New Zealand using RT-PCR and sequence analysis Ramneek, NL Mitchell, RG Mcfarlane New Zealand Veterinary Journal 53 (6), 457-461, 2005 | 15 | 2005 |
Electroretinography data from ovine models of CLN5 and CLN6 neuronal ceroid lipofuscinoses KN Russell, NL Mitchell, MP Wellby, GK Barrell, DN Palmer Data in Brief 37, 107188, 2021 | 11 | 2021 |
Practical implications of motion correction with motion insensitive radial k-space acquisitions in MRI MM Almuqbel, G Leeper, DN Palmer, NL Mitchell, KN Russell, RJ Keenan, ... The British Journal of Radiology 91 (1087), 20170593, 2018 | 8 | 2018 |
Natural history of retinal degeneration in ovine models of CLN5 and CLN6 neuronal ceroid lipofuscinoses SJ Murray, NL Mitchell Scientific Reports 12 (1), 3670, 2022 | 6 | 2022 |