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 2
Ultrastructural changes after special diet exposure in Gcdh^–/– mice. Electron micrographs of motor cortex from weanling (4w) WT (A and B) and Gcdh^–/+ (C and D) mice after 60 hours of protein diet show normal appearance including dendritic spines (white arrows) and mitochondria (black arrows). (E–G) Similar sections from weanling Gcdh^–/– mouse motor cortex after 24 hours of protein diet shows vacuolated neuron (E, thick arrows) identified by synapse (E, thin arrow, inset) and peripheral chromatin aggregation in the nucleus. (F) A large vacuole is noted (*) adjacent to an edematous dendrite, identified by synapse (white arrow) with disintegrating mitochondrion (black arrow). (G) Higher magnification of section similar to that in F shows postsynaptic (white arrows) swelling with enlarged and disrupted mitochondrion (black arrow). (H) Similar section from adult (8w) Gcdh^–/– mouse cortex shows vacuole formation after 60 hours of protein diet exposure. Scale bars: 1 μm.