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 3
Neuronal vacuolation after special diet exposure in Gcdh^–/– mice. (A) Semi-thin section of WT mouse CA3 hippocampus after 60 hours of protein diet shows normal appearance. (B) Similar section of adult Gcdh^–/– mouse after 60 hours of protein diet shows a few vacuoles (black arrows). (C) A similar section from a weanling Gcdh^–/– mouse after 48 hours of protein diet shows multiple vacuoles. (D) Confocal image of a section similar to that shown in C labeled with N52, GFAP, and DAPI shows vacuoles surrounded by N52 signal indicating neurofilaments (white arrows). (E–H) Acetylcholine transferase–positive neurons in the striatum of a weanling WT mouse (E) and weanling Gcdh^–/+ mouse (F) after 60 hours of lysine diet exposure show normal appearance. Development of vacuoles is prominent within the perikaryon and neuronal processes of a weanling Gcdh^–/– mouse (white arrows) after 48 hours of lysine diet exposure (G) and less prominent in adult Gcdh^–/– mouse after 60 hours (H). (D) N52, green; GFAP, red; and DAPI, blue. (E–H) ChAT, red; and DAPI, blue.