Table 2.
Brain specific protein concentrations in the cerebrospinal fluid from CLN6 affected sheep at different stages of the disease and age-matched controls (ng/l± sem)
Disease stage |
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---|---|---|---|---|
Aggressive neurodegeneration (5–10 months of age) |
Advanced terminal disease (18 – 32 months of age) |
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Marker | affected (n=6) a | control (n=12) a | affected (n=5) a | control (n=4) a |
NSE b | 1.1(0.2)c | 0.6(0.1) | 0.8(0.2) | 0.2(0.1) |
S100 b | 46.2(13.0)c | 34.3(4.6) | 7.1(0.9) | 4.0(0.7) |
GFAP b | 0.85(0.3) | 0.7(0.1) | undetectable | undetectable |
n= the number of animals sampled.
Immunoreactivity equivalent to human protein in μg/l (± sem).
One affected animal sample had NSE and S-100b concentrations of 7.16ng/l and 236ng/l respectively and was excluded as an anomaly. Including it in the data did not result in a significant difference between the affected and control means.