Mechanism of age-dependent susceptibility and novel treatment strategy in glutaric acidemia type I
J. Clin. Invest. William J. Zinnanti, et al. 117:3258 doi:10.1172/JCI31617 [Go to this article.]

Figure 5
Age-dependent brain biochemical changes with lysine diet exposure. (A) Chromatogram of acyl-CoA esters from brain extract of weanling Gcdh^–/– mice on normal or lysine diet shows large accumulation of acetyl-CoA (†) and depletion of free CoA (‡) after 48 hours of lysine diet. Abs, absorbance. (B) Free CoA, acetyl-CoA, and glutaryl-CoA changes in brain extract of weanling and adult Gcdh^–/– mice and heterozygous controls on normal or lysine diets. Mean ± SEM, *P < 0.01; **P < 0.001. n = 6 each group. (C) ATP, phosphocreatine (PCr), and α-ketoglutarate changes in cortex of weanling and adult Gcdh^–/– mice and heterozygous controls on the lysine diet compared with normal diet. Mean ± SEM, ^ΧP < 0.04; ^ζP < 0.02. n = 4 each group. (D) Glutamate, glutamine, and GABA levels in weanling and adult Gcdh^–/– mice and heterozygous controls on the lysine diet compared with normal diet. Mean ± SEM. n = 6 each group.