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. Author manuscript; available in PMC: 2015 Sep 1.
Published in final edited form as: J Inherit Metab Dis. 2014 Feb 3;37(5):735–743. doi: 10.1007/s10545-013-9675-2

Table 1.

Effect of NTBC treatment upon serum and brain amino acids in Pahenu2/enu2 mice.

Serum
(µM)
Brain
(nmol/g)
Amino acid Pah
−/−
Pah−/−
+NTBC
Mean
%
change
p
value
Pah−
/−
Pah−/−
+NTBC
Mean
%
change
p
value
Phenylalanine 2474 ± 99 1933 ± 109 −21.9 0.0009 693 ± 49 391 ± 20 −43.6 < 0.0001
Tyrosine 43 ± 3 256 ± 13 +595 < 0.0001 81 ± 15 110 ± 5 +35.8 0.0385
Tryptophan 63 ± 3 48 ± 4 −23.8 0.0045 20 ± 1 12 ± 1 −40.0 0.0004
Valine 252 ± 10 242 ± 11 −4.0 0.515 8 164 ± 9 111 ± 5 −32.3 0.0002
Leucine 176 ± 8 159 ± 11 −9.7 0.215 8 284 ± 16 193 ± 10 −32.0 0.0002
Isoleucine 116 ± 5 109 ± 9 −6.0 0.528 7 105 ± 6 80 ± 3 −23.8 0.0028
Methionine 61 ± 5 99 ± 18 +62.3 0.078 3 62 ± 4 51 ± 3 −17.7 0.0452
Histidine 69 ± 3 69 ± 3 0 0.922 9 114 ± 6 75 ± 4 −34.2 < 0.0001
Threonine 150 ± 9 171 ± 13 +14.0 0.096 6 261 ± 14 201 ± 8 −23.0 0.0022
Lysine 259 ± 32 252 ± 20 −2.7 0.419 9 312 ± 17 265 ± 12 −15.1 0.0210
Aspartic acid 13 ± 2 13 ± 12 0 0.4717 2073 ± 126 1895 ± 113 −8.6 0.1552

Serum and brain amino acid concentrations expressed as mean ± SE. p values calculated using one-tailed Students’ t test.

Data for which the difference between the means of NTBC-treated and untreated Pahenu2/enu2 mice was significant (p < 0.05) are displayed in blue Inline graphic.

Aspartic acid is displayed here as a reference amino acid that is not appreciably transported by the LAT-1 transporter.