Mechanism of age-dependent susceptibility and novel treatment strategy in glutaric acidemia type I
J. Clin. Invest. 117:11 doi:10.1172/JCI31617
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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). (EG) 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.