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Manifestation of neuronal ceroid lipofuscinosis in Australian Merino sheep: observations on altered behaviour and growth

By G. M. Cronin, D. F. Beganovic, A. L. Sutton, D. J. Palmer, P. C. Thomson, I. Tammen

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Neuronal ceroid lipofuscinoses (NCL) is an inherited neurodegenerative disorder in children. Presently there is no effective treatment and the disorder is lethal. NCL occur in a variety of non-human species including sheep, which are recognised as valuable large animal models for NCL. This experiment investigated the progressive postural, behavioural and liveweight changes in NCL-affected lambs, to establish practical, non-invasive biomarkers of disease progression for future preclinical trials in a CLN6 Merino sheep model. A flock of eight lambs at pasture was studied, with the observer blind to the disorder status. Three genotypes were compared: homozygous affected (NCL; n=4), clinically normal heterozygous (Carrier; n=2) and homozygous normal (non-carrier control (Normal); n=2). Direct observation during daylight and continuous accelerometer measurements over 72 h were used to quantify lamb posture and behaviour in 11 sessions between 26 and 60 weeks of age, conducted at 3-5 week intervals. There was a Genotype (G) * Age (A) interaction ( P=0.001) for liveweight of the lambs in the experiment, with NCL, Carrier and Normal lambs gaining 11.8, 16.5 and 23.4 kg, respectively, between 26 and 60 weeks of age. G * A interactions were also found for walking behaviour (means for NCL, Carrier and Normal genotype groups at 26 and 60 weeks, were 1.7 and 7.9%, 3.3 and 3.1%, and 2.5 and 1.9% of observations, P=0.008) and a composite variable of key behaviours identified in the principal components analysis ( P

Publication Title Applied Animal Behaviour Science
Volume 175
Pages 32-40
ISBN/ISSN 0168-1591
DOI 10.1016/j.applanim.2015.11.012
Language English
Author Address The University of Sydney, Faculty of Veterinary Science, Private Bag 4003, Narellan 2567, NSW,
  1. Animal behavior
  2. Animal models
  3. Animals
  4. APEC countries
  5. Australasia
  6. Australia
  7. Behavior and behavior mechanisms
  8. Bovidae
  9. Breeds
  10. Children
  11. Commonwealth of Nations
  12. Developed countries
  13. Diseases
  14. Evaluation
  15. Genetics
  16. Genotypes
  17. Grazing
  18. Humans
  19. Indicators
  20. Inheritance
  21. Interactions
  22. Lambs
  23. Mammals
  24. Markers
  25. Men
  26. models
  27. monitoring
  28. Oceania
  29. OECD countries
  30. pastures
  31. Posture
  32. Primates
  33. Ruminants
  34. Sheep
  35. ungulates
  36. vertebrates
  37. Wool
  1. peer-reviewed