Professor David Palmer
Faculty of Agriculture and Life Sciences
PO Box 84
Phone: +64 3 321 8136 (DD) or + 64 3 325 2811
Rare Disease Interest:
|Disease name||Researched (Human, animal)|
|Batten disease (neuronal ceroid lipofuscinoses, NCLs) Lysosome associated diseases; neurodegenerative diseases.|
This has led to an active interest in all lysosomal diseases, both animal models and the human diseases, and all aspects of them. This includes genetics, diagnosis, biochemistry, pathogenesis, pathology, clinical pathology and interactions, brain function and disruptions of it, putative treatments, family support and social effects.
|We have established two colonies of ovine models of Batten disease being the CLN5 and CLN6 forms in Borderdale and South Hampshire sheep, and study these as models of the human diseases.|
Field of research:
We research aspects of lysosomal storage disease with an emphasis arising from ovine models of Batten disease (neuronal ceroid lipofuscinoses, NCLs) following on from the diagnosis of two naturally occurring forms in New Zealand Borderdale (CLN5) and South Hampshire (CLN6) sheep. Breeding colonies have been established and provide animals and tissues for a range of investigations. Resources include live animals, fresh frozen tissues, neural cell cultures and perfusion fixed brain sections from all stages of disease development, from perinatal to end-stage disease. Investigations include genetics, biochemistry, cell biology and neuropathology with a current emphasis on the role of neuroinflammation in pathogenesis. Behavioural and electrophysiological studies are also underway to provide bench-marks for the assessment of the efficacy of putative therapies. These studies are being carried out at Lincoln and with groups led by Dr Stephanie Hughes at Otago University and Dr Imke Tammen at Sydney University making up BARN, and with BARN associates in Cambridge and King’s College, Professors Jon Cooper, Jenny Morton and Sir John Walker. They are integrated with studies of other animal models with the specific purpose of improving knowledge and opportunities for intervention in the human diseases.