. Author manuscript; available in PMC: 2010 Dec 1.

Published in final edited form as: Neurochem Int. 2009 Aug 5;55(8):783–788. doi: 10.1016/j.neuint.2009.07.012

Table 3.

Amino acid concentrations in the cerebrospinal fluid from CLN6 affected sheep at different stages of the disease and age-matched controls (μM ± sem)

	Disease stage
	Aggressive neurodegeneration (5 – 10 months of age)		Advanced terminal disease (18 - 32 months of age)
Amino acid	affected (n=10)^a	control (n=20)^a	affected (n=5)^a	control (n=4)^a

aspartate	2.5(0.2) ^b	2.4(0.2)	2.5(0.3)	3.2(0.4)
glutamate	2.6(0.4)	2.3(0.2)	3.8(0.4)	2.5(0.25) ^c
serine	68.2(6.2)	65.6(2.5)	60.6(6.7)	67.5(7.0)
glutamine	748.8(61.6)^c	635.6(19.1)	1008.3(139.5)	847.3(46.9)
glycine	24.1(3.1)^c	23.4(2.4)	19.9(1.4)	21.8(2.6) ^c
taurine	3.4(1.0)	2.5(0.3)	3.0(0.2)	6.1(2.8)
GABA	0.96(0.09)	0.99(0.05)	0.31(0.03)	0.24(0.04)

n= the number of animals sampled.

μM ± sem.

One control animal sample which had glutamate and glycine concentrations of 13.6μM and 199μM respectively was excluded as an anomaly. Including it in the data did not result in a significant difference between the affected and control means.