Axonal degeneration in paraplegin-deficient mice is associated with abnormal mitochondria and impairment of axonal transport
J. Clin. Invest. Fatima Ferreirinha, et al. 113:231 doi:10.1172/JCI20138 [
Go to this article.]
Figure 3Paraplegin-deficient mice show axonal swelling and degeneration. Semithin transverse sections of the spinal cord of
Spg7–/– mice at different ages. At 4 months, the spinal cord appears normal (
a). At 8 months, sporadic swollen axons are detected (arrowhead) (
b). Several swollen fibers (arrowheads) and degenerating fibers (arrows) are evident in the anterolateral funiculi (
c) and dorsal columns (
d) at 15 months. Note normal ventral roots in
b and
c. In a 24-month-old
Spg7–/– mouse, besides swelling (arrowheads) and degeneration (arrows), the density of myelinated axons is decreased, indicating significant axonal loss (
e). (
f) Semithin section of the optic nerve of a 19-month-old
Spg7–/– mouse showing a swollen axon (arrowhead). (
g) Immunostaining for neurofilaments in the derma of the distal hindlimbs of an 8-month-old
Spg7–/– mouse showing an axonal spheroid. Axonal swelling (arrowhead) and degeneration (arrows) in the sciatic nerve of
Spg7–/– mice at 19 months (
h) and 24 months (
i). Bar represents 40 μm in
a–
f and 20 μm in
h and
i.
