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

By Greg M. Cronin, Danai F. Beganovic, Amanda L. Sutton, David J. Palmer, Peter C. Thomson, Imke Tammen

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Abstract

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

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Tags
  1. Genetics
  2. Sheep